international cme & annual conference of highlights …odishahematology.com/11th...

6 - 8 S e p t e m b e r 2 0 1 9 , b h u b a n e S w a r , o d i S h a , i n d i a

Blood cancer services should Be led By haematologists

rK JenaPresident, Indian Society of Haematology and Blood Transfusion (ISHBT), Secretary Odisha Haematology

I extend my heartiest welcome to all the faculty (International and National), delegates and guests to this wonderful Temple city of Bhubaneswar. This city is

hosting International CME and 11th Annual Conference of Odisha Hematology on 7th and 8th September 2019 at May-fair Convention Centre, Bhubaneswar. A pre-conference workshop has been conducted on 6th Sep 2019 at IMS & SUM Hospital, Bhubnaeswar.

Haematological malignancies like Leukaemias, Myelo-mas, Lymphomas, Chronic Lympho-proliferative Disorders (CLPD) and Myeloma Proliferative Neoplasmas (MPNs) have witnessed tremendous advances both in diagnosis (both conventional and molecular haematology) and treat-ment which are very clearly unmatched in any other disci-pline of medicines. The space of developments, approval of new drugs, risk satisfaction, frequent changing standard of care (SoC), concept of patient specific protocol, meticulous interim monitoring and modification of therapy in between and subsequent/concurrent benefit of Bone Marrow Trans-plant procedure etc. have made the concept of pan-cancer specialists obsolete.

It is for these reasons, cancer special-ists are of 2 types: Treating Solid Tumors (Non Haematological Cancer) and Liquid Tumors called Haematological Cancers (Leukemias, Myelomas, Lym-phomas etc). Haematological cancer patients are best taken care by haematologist. This is a standard practice across the globe including many state-of-the-art institutions in India.

Recent positive developments like capping of price for cancer drugs, availabil-ity of diagnostic, medicine and supportive therapy includ-ing blood products in Odisha by State Government to all the patients free of cost has changed the primary objective of the therapy from “Palliative option of past to Curative intention at present”. Every patient in our state deserves to receive the standard of care; nothing less or nothing more.

This is only possible if these patients are taken care of by haematologists. This important point must be spread among patients, doctors, health care providers and all stakeholders.

ME

SS

AG

E

highlightsInherited Bone marrow failure

syndromes - Old & new tools in

diagnosis

Speaker: prof. Manoranjan

Mohapatra, aIIMS, New Delhi

Hemoglobinoptahy a global burden

Speaker: prof. kalpana Gupta,

Division of Haematology, Oncology

and Transplantation, University of

Minnesota, USa

haemato-logical can-

cer patients are Best taKen care By haematologists. this is a standard practice across

the gloBe

governor hails doctors for selfless service

OdIsha Governor Prof. Ganeshi Lal on Saturday hailed doctors for their selfless and determined service to the people

and society.Inaugurating the 11th annual conference of

Odisha Hematology here on Saturday, Prof Lal said, “when Sri Maa of Pondi-cherry is asked by a devotee about definition of a perfect doctor, she says a doctor has broad mind and generous heart, unflinching will, sturdy determination, inex-haustible energy and total trust in mission. Caste, creed, colour, cunningness, conspiracies and corruption never dare visit door of a doctor.”

He termed the doctor-patient relation-ship as a spiritual one. “Unless one understands what precious life of a patient is, one cannot treat the patient. The patient should have the full faith and belief in doctors and only then medicine can have some effect,” he said.

“Doctor is a soldier. Therefore all the dis-orders, all discordances, all disharmonies, all

distortions and all disruptions are nothing but phantom of the past. Doctor is a soldier, who van-quishes all these things,” the Governor added.

More than 300 doctors from Odisha, West Ben-gal, Jharkhand, Delhi, Bihar, North-East and

central India have gathered here to dis-cuss blood disorders and diseases,

share ideas on the latest advances in the diagnosis and patient care management.

Professor RK Jena, President of ISHBT and Secretary of Odi-sha Haematology, threw light on the importance of haematology

in the field of medical sciences. “There are many haematological

diseases like anemia, (50% of Indian population suffer from anemia), thalas-

semia, sickle cell disease, hemoglobinopathies, platelet disorders and haematological cancers like leukemia, myeloma and lymphoma. If you combine together, they will definitely outnumber the number of other diseases humans suffer at present.”

contd...p3

11th international cme & annual conference of odisha hematology inaugurated, over 300 doctors taKing part

the Burden of haematological

diseases far outweighs all other diseases

comBined gloBally at

present

laboratory hematologysum annual cme on2nd

odisha hematologyinternational cme & annual conference of

&

11th

s p o n s o r s

MYELODYSPLASTIC SYNDROME: CURRENT DIAGNOSTIC PRACTICES

DR. DEEPAk k MIShRA

P2

insi

de

P7 P8P3

INTEGRATED STRATEGIES TO IMPROvE SCD OUTCOMES

kALPNA GUPTA

HigH grade vs low- grade lympHoma - How TO TREAT

DR TUPhAN kANTI DOLAI

CONSTANT UPDATE & UPGRADATION kEY TO hOLISTIC CARE

DR PRIYANkA SAMAL

day2



&

11th


myelodysplastic syndrome: current diagnostic practices

new state health policy soon: health minister

MyelOdysplastIc syndrome (MDS) is a clonal hematopoietic stem cell disorder characterized by peripheral blood cytopenia, morphological dysplasia,

recurrent genetic aberrations and a risk of progression. MDS is more commonly considered as a disease of adults with a median age of 70 years at diagnosis. MDS in childhood is rare, but a unique entity.

Better understanding of the natural history of disease and development of newer therapeutic drugs has led to many modi-fications in classifications and prognostic scoring systems in the past 70 years, since this entity is known. With the advent of newer diagnostic tools many overlapping conditions have been identified and this has revolutionized the prognos-tic implications and therapeutic decisions in the recent era.

In the past three decades vari-ous classification systems with its updates have been published. In 2016, WHO classified MDS into six categories based on the num-ber of lineages involved, percent-age of blasts in peripheral blood and bone marrow, presence or absence of ringed sideroblasts and cytogenetics.

All the cases presenting with unexplained cytopenias should be worked up for MDS. Bone marrow examination, including aspirate for assessing morphological dysplasia and biopsy for determination of cellularity and architecture is important. Cy-togenetics plays an important role in determining the clonality and risk stratification.

The WHO classification subtypes provides comprehension for prognosis, but with use of cytogenetics and molecular tech-niques newer scoring systems have been used to prognosticate the patients with MDS. MDACC-MDS is another prognostication model which also incorporates the clinical characteristics of the patients and might be helpful in overlapping or progressing cases of MDS.

The current diagnostic practices intend to facilitate proper and timely diagnosis directing the appropriate therapeutic strategy.

OdIsha Government will soon come up with a new Health Policy to comprehen-sively address various issues of disease

burden, health services and infrastructure in the State, said Shri Naba Kishore Das, State Health and Family Welfare Minister.

Inaugurating the second Sum Annual CME on Laboratory Haematology on the eve of the 11th annual conference of Odisha Haematology on Fri-day, Shri Das said,”We are planning and working on the new policy in which health administration will become more responsive.”

Stating that seven new medical colleges had come up in the State after a gap of 52 years while six more were in the pipeline, the Minister said the Government was also working on a ‘Vision for 2025’.

The annual CME kicked off to a rollick-ing start with 150 delegates from dif-ferent institutions of Odisha taking part in the academic discussion.

Haematologists delivered talk on different aspects of cell counters, clinical perspectives, basic technological principles, haemocytomorphometry, under-standing of anaemia on automated erythrocyte analysis, WBC histo-grams, platelet counts, newer diagnos-tic parameter of sepsis, flow cytometry and quality assurance. Participants said the CME sessions were very informative, educative and interactive.

The Institute of Medical Sciences and SUM Hos-pital said it was in the process of establishing a state-of-the-art molecular diagnostic and research centre which will be a major boost for the treat-ment of blood cancer.

“It is going to be a major value addition to the diagnostic and prognostic work for the patients of the state,” Dr. Priyanka Samal, Head of the depart-ment of Clinical Hematology at the hospital, said

at the inaugural event.Dr. Samal said that the department of Clinical Hematology and Stem Cell Transplantation

of IMS and SUM Hospital was one of its kind which was evolving as a referral

centre for all kinds of hematological disorders.

Prof. Ashok Kumar Mohapatra, eminent neurosurgeon spoke on the occasion. Stating that there were 84 cancer patients per one lakh population in India against

the global figure of 64, he said healthcare could be further bol-

stered by trying to use the available resources and equipment.

“BMTs can be done with an expenditure of Rs. 1.2 lakh to Rs. 2 lakh while corporate hospitals were charging Rs. 15 to 20 lakh for the proce-dure,” Prof Mohapatra pointed out.

Prof. Gangadhar Sahu, Dean of IMS and SUM Hospital, Dr. Rabindra Kumar Jena, President of the Indian Society of Hematology and Blood Trans-fusion, Prof. Pushparaj Samantasinhar, Medical Superintendent of IMS and SUM Hospital and Dr. Debahuti Mohapatra, Head of department of Pathology at IMS and SUM Hospital also spoke on the occasion.

dr deepaK K mishraTATA Medical Centre, Kolkata, India

seven new medical colleg-es have come up

in the state after a gap of 52 years while six more

are in the pipeline

in pics

c o n f e r e n c e

newsletterPage#2

sum hospital is in the process of estaBlishing a state-of-the-art molecular diagnostic and research centre

all the cases pre-

senting with unexplained cy-

topenias should Be worKed up for mds.

cytogenetics plays an impor-

tant role

2nd sum annual cme on laB haematology




&

11th

new approaches to addressing thalassemia

integrated strategies to improve scd outcomes

the prevalence of beta thalassaemia varies across the world with endemic areas predominantly in South Asia, Middle East, North Africa and southern Europe.

The majority of non-endemic countries in Europe have less than 1000 patients with Italy reporting an estimated 6000-7000. A number of European countries including Germany have seen an exponential increase in thalassaemia following the wave of migration in 2015-2018 especially populations coming from high prevalent areas like Iran, Syria and North Africa.

Thalassaemia, though predominantly a problem of low-mid-dle income countries, is now attracting more attention for high income countries. It has seen the development of strategies to addressing prevention, treatment and recognition of com-plications from the disease, blood transfusion and co-existing infections.

Addressing the scourge of thalassaemia globally is a mul-tifaceted approach that has evolved through the implementation of widespread education and screening techniques, discussions and provision of prena-tal diagnosis and pre-implantation genetic diagnosis. The develop-ment of new therapies such as luspatercept and stotatercept which are involved in later red cell development and prevention of apoptosis to recent develop-ment in curative therapies such as bone marrow transplantation βoβo and non- βoβo thalassaemia.

New therapies and curative therapies will not be available or affordable to the majority of patients especially those living outside high income countries in the western world. The last two decades however have witnessed the twin ben-efit of blood transfusion therapies and effective iron chelation which remain the most effective intervention that have led the improvement in survival and quality of life.

Reviewing some of the reported patient cohorts including Ira-nian, Cyprus, Greek and Italian cohorts will address the impact of small additive steps on survival and patient related cohorts. We will discuss the disparity in the age survival comparing such end points as 10, 20, 30 year survival how these have con-tinued to evolve over the years.

Reporting more cohorts in Asia and Africa will encourage services in developing countries to adopt and share best practice within these regions because the lessons can be easily applicable locally. We will address ongoing challenges achiev-ing optimum chelation despite the fact that both single agent and combination chelation therapies are reported between the major agents such as Deferasirox, Deferiprone and Desferriox-amine.

Overall survival in thalassaemia major (transfusion depen-dent thalassaemia) is the key milestone in the assessment of patient related outcomes. Nevertheless advancement in the recognition of infections, cardiac iron , endocrine, metabolic complications and the management of co-morbidities becomes more apparent as patients survive into adulthood.

lInus Pauling first described Sickle Cell Disease (SCD) as a molecular disorder in 1949. Over half a century later, SCD not

only remains a scientific enigma but also afflicts millions of people worldwide.

The SCD is an autosomal recessive disor-der caused by a point mutation in the beta-globin gene, resulting in sickle hemoglobin (HbS). Under low oxygen HbS polymerizes to form rigid fibers and confers a sickle shape to the RBCs. Sickle RBC show reduced flexibility result-ing in erythrocyte membrane damage and hemolysis. The ensu-ing disease is characterized by hemolytic anemia and inflammation. Sickle RBC lead to the pathogenesis of number of organ-specific complications with enormous phenotypic variability.

Sickle RBC cluster together, occluding the blood vessels and impairing oxygen supply to the limbs and other organs, resulting in cumulative organ damage and acute painful episodes called “crises”. Micro- and macro-occlusions cause ischemic injury to virtually every organ/tissue including but not limited to the brain, heart, lungs, spleen, kidneys, bones, muscle, nerves, penis and retina. Recurrent episodes of crises and pain lead to increased hospitalization, morbidity and mortality, resulting in reduced lifespan of SCD patients.

A hallmark of SCD is intense pain that can start during infancy and increases in severity through-

out life, reaching levels considered higher than labor pain during pregnancy. SCD is accompa-nied by acute painful episodes (“crises”) super-imposed on chronic pain. The pathophysiology of SCD is compounded by inflammation, vascu-

lopathy, ischemia-reperfusion injury, organ damage and neuropathy, each of which

may contribute to pain. For example, behaviorally, “crises” result in

acute episodes of severe pain that appears with each consecutive vaso-occlusive event, whereas ensuing tissue and/or bone dam-age may lead to persistent pain.

In addition, inflammation in SCD may potentiate both episodic

and persistent pain. Clinical stud-ies using self-reported assessment of

symptoms in patient diaries show marked variability in pain presentation, apparently due to diverse factors including geographical location, sex, age and temperature, demonstrating the complexity of pain in SCD.

Opioids remain the mainstay for pain in SCD, which have liabilities of their own, and fear of addiction further obstructs their use even when needed. Therefore, integrative strategies are required that can improve the health and survival as well as improve symptom management in SCD. Some of these integrative strategies include diet modulation, mind-body exercises and acupunc-ture, which are cost-effective and user-friendly for resource limited settings with relatively large populations of SCD patients.

BaBa inusa Professor, Paediatric Haematology King’s College London Lead Consultant, Sickle cell and Thalassaemia Evelina London, Guy’s and St Thomas NHS Trust

Kalpna guptaUniversity of Minnesota

Medical School, USA

strate-gies include diet modula-

tion, mind-Body exercises and acu-

puncture, which are cost-effec-tive and user-

friendly

overall survival in

thalassaemia maJor is the Key milestone in the assessment of pa-

tient related outcomes

in pics

c o n f e r e n c e

newsletterPage#3

governor hails doctors

If you look at the development in medical scienc-es, haematology stands apart. The pace at which both clinical and laboratory haematology are developing, it is breathtaking. If you look at devel-opment of drugs in the stream, it is fast changing. There is a need for dedicated force of haematolo-gists and haematology departments in the country to provide standard care to patients, said Dr. Jena.

“It is a big concern at our haematology so-cieties that there are many states like Madhya

Pradesh, Chhattisgarh and Bihar where haematol-ogy department does not exist at all and in few states, haematology exists in few centres,” he said.

Among others, Prof Niranjan Tripathy, Presi-dent of Odisha Haematology, Dr. Gitanjali Bat-manabane, Director of AIIMS, Bhubaneswar, Amit Banerjee, Vice Chancellor of SOA University, H. P. Pati, Professor Haematology, AIIMS Delhi and Dr Priyanka Samal, Head of Haematology of SOA University spoke on the occasion.



&

11th


understanding anaemia from automated erythrocyte analysis

Blood cell counters : clinicians’ perspective

megaloBlastic anaemia: at a glance

MOst modern haematology analyzers are capable of performing white blood cell (WBC) differential counts as well as providing extended information on the pres-

ence of immature granulocytes, blasts of various lineages and gauge interference by nucleated red cells in the WBC count. On the other hand, automated red blood cell (RBC) analysis has been mostly limited to the RBC count, red cell indices, reticulo-cyte counts and a few volume-based parameters.

Concerns about KCN toxicity have led to development of non-hemoglobinocyanide (HiCN) methods to measure Hb which are also less susceptible to interference from conditions that spuriously elevate HiCN-based Hb measurement via increased turbidity like leukocytosis, hypertriglyceride-mia or paraproteinemias.

This RBC size frequency distribution histogram typi-cally displays a symmetrical or Gaussian shape. MCV and RDW are directly derived from this curve. This histogram can show several abnormalities in various disease settings and can indicate the presence of fragmented RBC, micro-spherocytes, platelet clumps, macrocytes, small agglutinates or reticulocytes. Red cell distribution width (RDW-CV and RDW-SD) are measures of the dispersion of data around the mean, thus measures degree of anisocytosis. More-over, because it is a direct measure across the RBC distribution curve, one can roughly estimate the spread of the distribution of cells by examining the histogram.

Automated reticulocyte counting involves the use of a nucleic acid binding (fluorescent/non-fluorescent) dye like thiazole or-ange or new methylene blue. As well as yielding a percent and absolute reticulocyte count, the immature reticulocyte fraction (IRF) can be measured as the sum of the reticulocytes with me-dium and high fluorescence. Features of the combined absolute reticulocyte count and IRF are characteristic of different disease states and may be clinically useful in classifying anemia. Other reticulocyte parameters are reported in sphered reticulocytes similar to those for the sphered mature RBCs. The hemoglo-bin content of reticulocytes (CHr), measured on the Siemens Advia instruments, is a sensitive and specific indicator of iron deficiency, monitoring iron therapy in renal failure patients on chronic dialysis and monitoring for the onset of iron deficiency following treatment with erythropoietin.

BlOOd cell counters have undergone tremendous technological revolution and now are the cornerstones of any diagnos-

tic laboratory to provide extremely precise, timely and interpretable inferences for investigations raised by the clinicians.

Along with classical parameters such as haemoglobin, total and differ-ential leucocyte counts, platelet counts and red blood cell (RBC) indices, many new parameters have come up which provides detailed information of a cell population and also gives clues to various clinical conditions. The immature reticulocyte fraction (IRF), mean reticulocyte volume (MRCV), mean reticulocyte hemoglo-bin content (CHr), the immature platelet fraction (IPF), RBC distribution width (RDW) and platelet indices are few to name which the clinicians and pathologists have started using in decision making.

Newer parameters act as surrogate to many clinical conditions and these can be used in mak-

ing clinical decisions, however with caution. With the immense improvement in software technology the automated cell counters are optimized to give a suspect flag or a warning signal. With proper clinical judgement and analysis, the counters

can be customized with systematic update to provide appropriate and required

alerts. This advancement has not only brought down the turnaround but

also allows interpretation of the disease status. As a result, haema-tologists can devote more time to the smear examination of the samples with specific flags.

Although the most advanced blood cell counters can be tailor-

made according to the institutional requirements, proper quality checks

and standardization are key to accurate results. Blood cell counter parameters are the important hematological tests used routinely in clinical decision making. A clinician must identify the power as well as restrictions of the modern blood cell counters for better clinical use in ben-efit of the patient.

MegalOBlastIc anaemia is one of the common causes of anaemia and the treatment is not only simple but also

easily affordable. If left untreated, it can lead to morbidity due to both anaemia and accom-panying neuropsychiatric manifestations. Though sometimes it masquerades as hemolytic anaemia, pancytopenia mimicking MDS may mislead too. A correct diagnosis and prompt therapy can lead to a complete recovery.

Megaloblastic anemia de-scribes a disorder characterized by distinctive hematopoietic cell morphology, due to defective DNA synthesis and prolonged S-phase of cell cycle. Nutritional deficiency is the most common cause of megaloblastosis. The etiology of the anaemia includes Vitamin B12, folate and copper deficiency and a wide range of drugs that interfere with purine and pyrimidine metabolism.

Many patients remain asymptomatic and detected on routine evaluation revealing a raised mean corpuscular volume (MCV). Common

presenting symptoms are anaemia, glossitis and angular stomatitis. Skin hyperpigmentation also occurs which are reversible. Cobalamin deficien-cy can also result in premature graying of hairs.

Vegetarians with cobalamin neuropathy having cognitive impairment in nearly 50% of cases

has also been reported in India. Another study in India showed presence of

neurological symptoms at the time of presentation in 33% of children with megaloblastic anaemia.

It is diagnosed through laboratory evaluation of CBC and PBS. The classical findings are reduced hemoglobin, increased

MCV (110 to 130 fl) with raised red cell distribution width (RDW).Treatment of megaloblastic anaemia

is with parenteral cobalamin and oral folate. But it should be started only after establishing the diagnosis and the type of vitamin deficiency. However, in severely ill patients, early treatment even before establishing the diagnosis may be needed. Blood transfusion is usually not required and should be avoided unless patient is having features of heart failure.

dr prashant sharmaPGI, Chandigarh

dr deepaK K mishra

TATA Medical Centre, Kolkata, India

dr pritish patra

Associate Professor, Haematology, IMS & SUM

Hospital, Bhubaneswar

identify the power as well as restrictions of

modern Blood cell coun-

ters for Better clinical use

many pa-tients remain asymptomatic

and detected on routine evaluation revealing a raised

mean corpuscu-lar volume

advia in-strument, is

a sensitive and specific indicator of iron deficiency,

monitoring iron therapy in renal

failure patients

in pics

c o n f e r e n c e

newsletterPage#4



&

11th


thromBocytopenia in the intensive care unit

complete Blood counts – more than Just numBers

disseminated intravascular coagulation

thrOMBOcytOpenIa is generally defined as platelet counts <150 × 109/L and represents a common laboratory finding in intensive care unit (ICU) patients.

Nearly 50% of patients present with thrombocytopenia at some point of time in their ICU stay with 20% of them developing severe thrombocytopenia (<50 × 109/L).

The mechanisms contributing to thrombocytopenia in the ICU include pseudothrombocytopenia, hemodilution, plate-let consumption, decreased platelet production, increased sequestration of platelets, and immune-mediated destruction of platelets.

Sepsis accounts for approximately 50% of all thrombocytopenias in ICU. Trau-ma-induced coagulopathy together with hemodilution due to massive transfusion of red blood cells and plasma units is also a common cause of thrombocytopenia in the ICU.

Identifying and treating the underlying cause or causes of thrombocytopenia in the critically ill is the crucial step for successful management. Current guidelines rec-ommend platelet transfusions to maintain the platelet count >50 × 109/L in trauma patients and >100 × 109/L in patients with ongoing bleeding and/or traumatic brain injury.

In patients with immune-mediated thrombocytopenia like ITP and DITP, however, platelet transfusions should be restricted to those with serious or life-threatening bleeding. The same ac-counts for patients with acute HIT or TTP.

A recent expert recommendation suggests prophylactic transfusion of platelets in patients with severe sepsis at a thresh-old of ≤10 × 109/L, and if the patient has a significant risk of bleeding, a threshold of ≤20 × 109/L is recommended. Trans-fusion of single donor platelet (SDP) or apheresis platelet is always preferred as they provide optimized therapeutic benefit to the patient and reduces the chances of platelet refractoriness.

MultI-paraMeter cell counters can measure, derive and/or calculate CBC parameters, WBC types and reticulo-

cytes. Most possess auto-sampling with barcode identification, system status monitoring, and computer-enhanced data analysis with computer storage of patient and control data capabilities.

Cell distribution graphs, as well as sample and instrument flagging systems, are standard features with visual or audible alarms. Obviously, the instruments have moved on from being “Cell Counters” to versatile systems capable of providing information either by actual testing, e.g. fluorescence, or by calculation algorithms.

Several new blood cell param-eters are potentially useful in the differential diagnosis of various diseases. Newer instruments have entered the analyzer market, in alpha-betical order of the manufacturers, Sap-phire (Abbot Diagnostics Cell Dyn), LH750 and UniCel DxH 800 (Beckman Coulter), Pentra (Horiba Medical), BC 6800 (Mindray), Advia (Sie-mens), and XE and now XN series (Sysmex).

Many new parameters have been introduced some of which include nucleated red blood cells, fragmented red cells, % hypochromic red blood cells, low hemoglobin density, red cell distribu-tion width, immature reticulocyte fraction and reticulocyte haemoglobin content for Red Blood Cells; haematogenic progenitor cells, immature granulocyte, white cell volume, conductivity and scatter, high fluorescent lymphocytes and Neut-X

& Neut Y for White Blood Cells;immature platelet fraction, platelet distribution

width, mean platelet volume, plate-lets/large cell ratio, mean platelet content, platelet dry mass and plateletcrit for Platelets.

The amount of information available from automated hae-matology analyzers has exploded

during the past 20 or more years, reflecting tremendous develop-

ments in technology. The new ad-vanced clinical parameters enhance the

routine complete blood cell count in all cell lineages to include leukocytes, erythrocytes, and platelets. Incorporation of these parameters has documented value in improving patient outcomes.

dIsseMInated intravascular coagu-lation (DIC) is an acquired syndrome characterized by widespread activation

of coagulation leading to fibrin deposition in the vasculature, organ dysfunction, consumption of clotting factors and platelets, and life-threat-ening hemorrhage.

DIC is provoked by several under-lying disorders like sepsis, cancer, trauma, and pregnancy complicat-ed with eclampsia or other calam-ities. The main trigger stems from widespread over-expression of TF in blood circulation, which leads to excessive thrombin gen-eration, low levels of natural coagu-lation inhibitors, mainly AT and PC, imbalance of the equilibrium between TFPI and TF, fibrinolysis impairment or acti-vation, fibrin deposition in microvasculature, and consumption of platelets and clotting factors.

The clinical manifestations of DIC depend on the underlying disease, individual predispos-ing factors, and the phase of the degradation of defense mechanisms. The inefficiency of natural anticoagulant pathways in general precedes the profound deficiency of clotting factors. The high

mortality rate of 20% to 50% in DIC drives the need for early diagnosis and aggressive treat-ment of both the underlying disease and the coagulation abnormalities. Early diagnosis of DIC is of major importance for restoration of blood

coagulation abnormalities and patient’s survival.

Therapeutic strategies specific for DIC aim to control activation of

blood coagulation and bleeding risk. Although there is very lim-ited data to support the efficacy of FFP, fibrinogen concentrate, PCCs, and platelet transfusion in controlling DIC, these are still the

standard of care in patients with severe DIC-associated hemorrhage.

Transfusions are generally monitored by routine clotting tests such as PT, aPTT,

and fibrinogen. Assays based on thromboelas-tography may aid in the identification of patients with hyperfibrinolysis as well as early diagnosis of hypofibrinogenemia and severe clotting factor deficiency. The analysis of clinical trials and the pathophysiologic mechanism show that DIC is a heterogeneous condition requiring individualized treatment.

dr sudipta seKhar dasProfessor of Transfusion Medicine, Apollo Hospital Education & Research Foundation (AHERF), India

dr anil handoo

BLK Super Speciality Hospital, New Delhi

dr deBasis BanerJee

Consultant Pathologist, DRS Trivedi & Roy

Diagnostic Lab,Kolkata

amount of information

availaBle from au-tomated haematol-

ogy analyzers has exploded in the

past 20 years

dic is provoKed By

several underly-ing disorders liKe

sepsis, cancer, trau-ma, and pregnancy

complicated with eclampsia

identi-fying and

treating the un-derlying cause of

thromBocytopenia in the critically ill is the crucial

step for suc-cess

in pics

c o n f e r e n c e

newsletterPage#5



&

11th


flow cytometric immunophenotyping

plasma cell dyscrasia- current diagnosis

lymphoproliferative disorders:mimicKers of lymphoma

FlOw cytometric immunophenotyping remains an impor-tant tool for the diagnosis, classification and monitoring of hematologic neoplasms.

Flow cytometry have ability to identify different normal cell populations and recognize phenotypic aberrancies, even when present in a small proportion of the cells analyzed. Flow cytometric immunophe-notyping evaluates individual cells in suspension for the presence and absence of specific antigens.

It allows detection of abnormal cells through identification of antigen expression that dif-fers significantly from normal. It also helps in identification of cells from different lineages and determination of whether they are mature or immature. Flowcytometric immunophenotypic analysis also tells us about if any other additional studies that might be of diagnostic value such as immunohistochemistry, conventional cytogenetic, fluorescence in situ hybridization (FISH), and molecular diagnostic studies; and finally the provi-sion of immunophenotypic information that might be of addi-tional prognostic value with potential directed therapy.

dIsOrders characterized by abnormal proliferation of immunoglobulin-pro-ducing cells and abnormal production of

immunoglobulin (M-protein) represent the spec-trum of diseases called plasma cell dyscrasia, synonyms with paraproteinaemia , or monoclonal gammopathy. It includes Multiple myeloma, Plasmacytoma (Medllary / extra-medullary), Waaldenstrom’s macro-globulinemia (lympho-plasmacytic lymphoma), plasma cell leukemia, heavy chain diseases, light chain AL-amyloidosis, monoclonal gammopathy of uncertain sig-nificance, osteosclerotic multiple myeloma (POEMS syndrome).

In view of varied disorders in-cluded in Plasma Cell Dyscrasia , the clinical presentation and investigations also differ to a large extent except the tests for monoclonal paraprotein.

Myeloma is suspected when patient presents with pathological fracture with trivial impact/ repeated fractures/ bone pain/ compression frac-ture, peripheral neuropathy, chronic renal disease, normocytic anaemia without explainable cause in elderly patient, high serum globulin, renal insufficiency, hyperviscosity and infection such as

pyelonephritis and pneumonia.Bone marrow aspirate & biopsy in gamopathy is

indicated when suspecting myeloma/ Amyloido-sis/ Waldenstrom’s macroglobulinemia. Immu-nohistochemistry is also useful to demonstrate quantity of plasma cell involvement more precise-

ly by CD138. Amyloid deposit also can be shown on marrow biopsy by Congo-red.

To differentiate mature plasma cells from myeloma cells flow cytometry

is commonly used.Clinical presentations in

Amyloidosis are very different from myeloma. Most common are nephrotic syndrome, congestive cardiomyopathy, sensorimotor

and/or peripheral neuropathy.The most important test for

Amyloidosis is tissue demonstration of tissue amyloid deposit of amyloid. Histo-

logical diagnosis, IHC for Amyloid P component, bone marrow aspirate, immunofixation of blood and urine and serum free light chains are part of diagnosis.

For diagnosing Light Chain Deposit Disease (LCDD), Heavy Chain Deposit Disease (HCDD) and WM/Lymphoplasmacytic Lymphoma various diagnosis methods are used.

lyMphOprOlIFeratIve disorders (LPDs) refer to several conditions in which lymphocytes are produced in excessive

quantities. Traditionally, the lymphoproliferative lesions are classified into two distinct categories benign (reactive) and malignant.

With the advancement in diagnostic modalities and greater understand-ing of the biology of lymph node pathology, grey zone of the two, atypical lymphoproliferative lesions (ALP) have been better described. Currently, however, no definitive classification system is available for ALP.

It is defined as the effaced architecture of lymphonode but not fulfilling the criteria of malignancy. Usually it has benign clinical course but sometime it may progress to malignant lesions in due course of disease. These lesions usually have discrepancies in the pathological feature and clinical behaviour.

The etiology of ALP includes infectious HIV, HHV, EBV and Toxoplasma; auto immune Rheu-matoid arthritis, SLE; drugs like cabamazepine,

dilantoin and immunosuppressive drugs; along with unknown aetiology like Castleman disease, Rosai Drofman disease or Kikuchi disease.

The worrisome histological findings include ef-facement of lymph modal architecture, large area

necrosis, abundant eosinophils and atypical cells with prominent nucleoli. General-

ized lymphadenopathy with hepa-tosplenomegaly and associated

B symptom indicates malignant aetiology.

The current proposed patho-genesis is polyclonal activation of lymphocytes to antigenic stimuli. These lesions may share few mo-

lecular features with lymphomas but lack the signature genetic altera-

tions which prevent the lesion to fully develop lymphoma.

A high degree of suspicion and multimodality approach like immunohistochemistry (CD3, CD20, CD4, CD8, CD30, EBV LMP, PAX5, CD79a, Cd68 Kappa, and Lambda), EBER in-situ hybridization, serology, viral marker, genetics and detailed clini-cal examination is required in order to accurately diagnose these entities.

dr Kiran ghodKeKokilaben Dhirubhai Ambani Hospital, Mumbai

dr hp patiProfessor, Haematology,

AIIMS, New Delhi

dr saumyaranJan mallicK

All India Institute of Medical sciences, New Delhi

in plasma cell dyscrasia , the clinical

presentation and investigations dif-fer to a large ex-

tent

with advances in

diagnostic mo-dalities, atypical lymphoprolifera-tive lesions (alp) have Been Better

descriBed

flow cytometry

have aBility to identify different normal cell popu-lations and recog-

nize phenotypic aBerrancies

in pics

c o n f e r e n c e

newsletterPage#6



&

11th


chronic itp-do we have answers in 2019?

IMMune thrombocytopenic purpura (ITP) is an immune mediated disease of adults and children characterized by decrease in platelet

count (<1lakh/cumm) and manifesting pheno-typically with variable bleeding symptoms. The incidence of primary ITP in adults is 3.3 cases per 1 lakh adults per year. Secondary ITP comprises 20% of all ITP cases.

Understanding of the pathophysiolo-gy of ITP has significantly improved. It is now clear that Primary ITP is an acquired immune disorder where thrombocytopenia results from destruction of platelet by antiplatelet antibodies, impaired megakaryocytopoiesis as well as T-cell–mediated destruction of platelets, with each pathologic mechanism playing varying roles in each patient.

Diagnosis of ITP is generally made by review of peripheral smear and evaluation of history and examination of the patient. The IWG has recom-mended a few additional tests which includes H

pylori, HIV, and hepatitis C as well as a direct anti-globulin test and blood type. The ASH guidelines recommend the same except H pylori which they recommended for some geographic areas only. The recommendations from IWG is to do bone marrow examinations in patients 60 years old with newly diagnosed ITP, whereas the ASH guidelines

suggest that bone marrow may not be necessary in any patient.

Corticosteroids, intravenous im-munoglobulin and anti-D immune globulin are the drugs used in newly diagnosed ITP. For patients with chronic ITP three main classes of therapies available that include splenectomy, rituximab

and thrombopoietin receptor ago-nists (TPO-RA).Novel approaches and newer

therapies like introduction of avatrom-bopag; combination treatment strategies: dexa

+ rituximab; dexa + rituximab + cyclosporine; recombinant human TPO with dexa, fostamatinib and rozanolixizumab have come up.

prof. maitreyee Bhattacharyya

Professor and Director IHTM, Kolkata

& dr Karuna Jha

corticoste-roids, intrave-

nous immunogloB-ulin and anti-d

immune gloBulin are used in newly

diagnosed itp

in pics

c o n f e r e n c e

newsletterPage#7

high grade vs low- grade lymphoma - how to treat

lyMphOMas are a heterogenous group of malignancies of B cells, T cells and rarely, natural killer cells that usually originate in

the lymph nodes, but may affect any organ of the body. They are broadly divided into Non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL).

According to clinical behaviour, the NHL are divided into indolent (low grade) and aggressive (high grade) lymphoma. Low grade cancer cells are well differenti-ated and act much like normal cells. They tend to grow slowly and have spread to other parts of the body by the time they are di-agnosed.High grade cells are poorly differentiated and gorw fast, needing immediate treatment.

Lymph node biopsy and histopathology and immunochemistry (IHC) is essential before initiating treatment. Stage of the disease, prog-nostic scoring systems, age and co-morbidities and performance status have to be considered

before deciding any form of therapy. Prototype of low grade lymphoma is follicular and high grade lymphoma is diffuse large cell lymphoma.

Treatment of follicular lymphoma has changed radically after introduction of rituximab. Other

treatment regimens include oral chloram-bucil, fludarabine or bendamustine or

combination chemotherapies with cyclophospamide vincristine, and

prednisolone; or anthracycline-containing regimens as CHOP.

Diffuse large B-cell lymphoma is localised in 25% of patients. Before 1980, radiotherapy was given to the affected region alone.

Subsequently, radiation wascom-bined with combination chemother-

apies with cyclophospamide vincris-tine, and prednisolone or CHOP. The most

important advance in treatment was addition of rituximab to chemotherapy. Addition of rituximab to CHOP-like chemotherapy has shown significant increase in overall survival rates from 52% to 78% in randomised trials.

dr tuphan Kanti dolai

Prof & Head, Haematology dept. NRS

Medical College and Hospital, Kolkata

the most important

advance in treat-ment of diffuse

large cell lympho-ma was addition of rituximaB to che-

motherapy and chop

sunday, s e pt ember 8 , 2 0 1 9

S c i e n t i f i c P r o g r a m m e

11th international cme & annual conference of odisha hematology & 2nd sum annual cme

on laboratory hematology

08:30 - 09:30 am: ISHBT STaTe qUIz fINal 08:30 - 09:30 am: SeSSIONS 5 MISCellaNeOUS:Inherited Bone marrow failure syndromes - Old & new tools in diagnosisSpeaker: • Prof. Manoranjan Mohapatra, AIIMS, New DelhiChairperons:• Prof. Bhagirathi Dwibedi• Pranati Mohanty• Mrutyunjay Dash• Smita Mohapatra09:55 - 10:40 am: MISCellaNeOUSpanel Discussion: Meet the spleen-the strange little organ that can multiplyModerator:Prof. Pranatar CHakrabarti, Fortis Hospital Kolkatapanelists:• Prof. L K Meher• Prof. Niranjan Mohanty• Anupam Chakrapani• Gurmeet Singh• Mihir Mohapatra10:40 - 11:00 : HIGH Tea11:00 - 11:25 am:SeSSION 6Global Session on Hemoglobinopathy:Hemoglobinoptahy A global burdenSpeaker: • prof. kalpana Gupta,Division of Haematology, Oncology and Transplantation, University of Minnesote, USAChairperson:• Prof. Sidhartha Das• Prof. CBK Mohanty• Prof. J N Behera• Prof. Arakhita Swain11:25 - 11:55 amGlobal Session on Hemoglobinopathy:

Survival trends in ThalassemiaSpeaker: • prof. Insua Baba, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Trust Vice Chairman, Sickle Cell Cohort ResearchChairperson:• Prof. Sidhartha Das• Prof. CBK Mohanty• Prof. J N Behera• Prof. Arakhita Swain12:00 - 12:25 pmGlobal Session on Hemoglobinopathy:Pain Phenotypes and implications in management of Sickle Cell DiseasesSpeakers:• prof. Deepika Darbari,Children’s National medical Center, Center for Cancer and Blood Disorders, USAChairperson:• Prof. Kashinath Padiary• Prof. Prashant Saboth• Prof. Jayashree Rath• Dr. Dinabandhu Sahoo• Dr. Lingaraj Pradhan12:30 - 01:00 pmGlobal Session on Hemoglobinopathy:Control of Hemoglobinopathy in India- The relevance of Odisha Model Speaker:• prof. alok Srivastava,CMC, VelloreChairperson:• Prof. Kashinath Padiary• Prof. Prashant Saboth• Prof. Jayashree Rath• Dr. Dinabandhu Sahoo• Dr. Lingaraj Pradhan1:00 - 2:00 : lUNCH2:00 - 3:00 pM : flash talk by pG Students3:00 - 4:00 : Intersting discussions from different institutions of Odisha4:00 pm : Veledictory & Tea



&

11th

ME

SS

AG

E


constant update & upgradation Key to holistic care

I welcome all delegates, our esteemed international and national faculty, fel-low haematologists, authorities dealing

with overall management of blood diseases, researchers and partners to the 11th Interna-tional CME and Annual Conference of Odisha Haematology in Bhubaneswar.

Haematology has been an important branch of medical science. It not only deals with blood disorders but also with various heart diseases such as strokes, high blood pressure and a host of genetic ailments. Haematologists handle diag-nosis, treatment and overall management of blood disor-ders ranging from anemia to blood cancer

At a time when the West boasts of very high success rates in tackling haematological malig-nancies, similar success eludes us. It is mainly because two third of our people suf-fering from blood disorders are referred late and haematologists are left with little choice to plan treatment in the advanced stage. Apart from raising awareness among general public, practising physicians need to be very care-ful so that patients are referred at very early stage and can avail proper treatment.

The most challenging part of treatment is the infections with MDR (multi drug resis-tance) organisms. It is important that, we phy-sicians must avoid antibiotic use and abuse.

The Government has provided free blood transfusion and chelation therapy to thalas-semia patients and has also taken steps for arranging stem cell transplantation at very minimal cost, which is the only cure for these patients.

We should aim to have a thalassemia free India. It requires mass education and

awareness in premarital and prenatal diagnosis on a massive scale. It

will help reduce the burden of thalassemia on society. More-over, evidence-based therapy should be followed by physi-cians for achieving a uniform approach to treating blood disorders.

Laboratory haematology is an integral part of this branch

because without an accurate diagnosis it is not possible to start

any therapy. Indian pharmaceutical com-panies have also extended their support by manufacturing new drugs at affordable price, which will prove to be a boon for developing country like India.

We look forward to provide the best pos-sible care in the field of haematology by up-dating and upgrading ourselves through such scientific interactions with experts from across the country and globe.

I thank all the members of the organizing committee, participants and the volunteers for making this event a great success.

dr priyanKa samal

Organizing Secretary

evidence-Based therapy should Be fol-

lowed for achiev-ing a uniform approach to

treating Blood disorders

c o n f e r e n c e

newsletterPage#8

in pics

s p o n s o r s

international cme & annual conference of highlights …odishahematology.com/11th...

Documents