diseases of wbc

Diseases of WBC & Lymph Nodes

Pathology-B Lab Ravi A Patel

M++Hodgkin’s Disease-Spleen

Non-Hodgkin’s disease

Multiple Myeloma

TB- Lymph node

Papillary carcinoma metastatic to lymph node

• What is it ?

– NHL represents a monoclonal proliferation of lymphoid cells and be of B-cell origin(70 %) or T-cell origin(30 %).

-- The incidence of this tumour increases with age.

-- Clinical treatment depends on the grading(reflection of proliferation rate)- Higher the grade, higher the proliferation rate.

• Organ involved- Lymph node

• Extra nodal involvement is common– Bone marrow(more common 60% in low grade than high grade disease), gut, thyroid, lung, skin, testis, Brain and rarely bone.

• Extra nodal involvement is more common in T-cell disease

• Clinical manifestation:--- Lymph node enlargement which may be associated with systemic upset – weight loss, sweats, fever and

itching.

-- Compression syndromes may occur– GUT obstruction, ascites, superior vena caval obstruction and spinal cord compression.

-- Hepatosplenomegaly may be present.

• Diagnosis:- – Routine bone marrow aspiration with trephine(surgical instrument used to make hole in bone)

– Immunophenotyping of surface antigen to distinguish T & B cell tumours.

– Immunoglobulin determination

– Measurement of uric acid level(some very aggressive high grade NHLs are associated with very high urate levels, which can precipitate Renal failure when treatment is started)

– HIV testing(Appropriate only if risk factors are present)

• Management:- Asymptomatic patients may not require therapy. Indication of treatment include marked systemic symptoms, lymphadenopathy(causing discomfort), bone marrow failure or compression syndromes.

– Low grade NHL – following are the options• Radiotherapy- used for localized stage 1 disease which is rare.• Chemotherapy(chlorambucil- most patient respond to this) – Main stay therapy• Monoclonal antibody therapy- Rituximab showed improvement in 60% of the

patients.• Marrow Transplantion

– High Grade NHL- patients with high grade NHL need treatment at initial presentation• Chemotherapy(90 % of the patients need this) mainstay combination

intravenous therapy(Cyclophosphamide, doxorubicin, vincristine & prednisolone)• Radiotherapy• Monoclonal Antibody therapy-Rituximab(increases the survival rate in

combination with the Chemotherapy.• Marrow Transplantation

Majority of theTumour cellsHave the Appearance ofSmallRound Lymphocytes.

But they are irregularAnd have cleaved Nuclear outlines

HPO

Prolymphocyte(large cell)With a centrally Placed nucleolus

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LPO

DiffuseNodal Architecture

– It is malignancy of B-cell origin.

– Histologic hallmark:- Reed sternberg cells(large malignant cells of B-cell origin)

• Clinical Manifestation

– Painless rubbery lymphadenopathy, usually in the neck or supraclavicular fossae, may fluctuate in size..

– Young patients with Nodular sclerosing may have large Mediastinal mass which are surprisingly asymptomatic but may cause dry cough and some breathlessness.

– Hepatosplenomegaly may be present.

– Spread is contiguous from one node to other.

– Rare extra nodal involvement.

• Diagnostic investigation:-– Full blood count may be completely normal.A normochromic,normocytic anaemia

may be present together with lymphopenia which is a bad prognosis factor.

– ESR may be raised

– Renal function tests are required to ensure function is normal prior to treatment.

– Liver function test may be abnormal.

– Chest X-ray may show mediastinal mass

– CT scan of chest and abdoment to permit staging.

– Lymph node biopsy.

• Management

– Treatment options include Radiotherapy, chemotherapy or a combination of both.

WHO pathological classification & incidence of Hogkin lymphoma

Type Histology Incidence

Nodular lymphocytePredominant

Hodgkin lymphoma

Numerous mature looking lymphocytes surround

scattered large pale staining lymphocytic

variants(popcorn cells)

5%

Classical Hodgkin

Lymphoma

Nodular sclerosing(In young patients)

70%

Mixed cellularity(Lab slide)

20%

Lymphocyte rich 5%

Lymphocyte depleted Rare

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Apart From theRS cellsOther cellular Infiltrates like Lymphocyte arepresent

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Clinical differenceHodgkin lymphoma Non-Hodgkin Lymphoma

More often localized to single axial group of cervical, Mediastinal, Para aortic nodes

More frequent involvement of multiple peripheral nodes

Orderly spread by contiguity(step wise fashion characteristically)

Non contiguous spread

Mesenteric nodes and waldeyer ring rarely involved

Mesenteric nodes and waldeyer ring commonly involved

Extra nodal involvement uncommon Extra nodal involvement common

NON

HODGKINS

HODGKINS

100x

40x40x

20x

• What is it ?– It is a malignant proliferation of plasma cells.

– Normal plasma cells are derived from B-cells and produce immunoglobulins which contain heavy and light chains.

– Normal immunoglobulins are polyclonal which means that a variety of heavy chains are produced and each may kappa or lamba type.

– In Myeloma plasma cells produce immunoglobulin of a single heavy and light chain(monoclonal protein called Paraprotein)

– In some cases only light chain is produced and this appears in the urine as Bence jones protenuria.

• Pathology:-

– Majority of the malignant plasma cells are present in the bone marrow(although some are also present in circulation)

– The malignant plasma cells produce cytokines which stimulate osteoclasts and result in net bone absorption. The resulting lytic lesions cause bone pain, fractures and hypercalcemia.

Clinical Manifestation • Bone pain/Fractures due to lytic lesions

• Spinal cord compression– Bony collapse– Extradural mass

• Paraproteinemia– Immune paresis– Bence jones protenuria

• Renal failure– Paraprotein deposition– Hypercalcemia– Infection– Amyloid

• Amyloid

• Hyperviscosity- Retinal bleeds,Bruising,heartfailure,cerebral ischemia.

Diagnostic investigation

• Diagnosis of Myeloma requires one of the following criteria

– Increased malignant plasma cells in the bone marrow

– Serum and/or urinary paraprotein

– Skeletal lesions.

Management• If patients are asymptomatic treatment may not be required. If

symptomatic then measures below should be considered.

– Immediate support

• High fluid intake to treat renal impairment and hypercalcemia

• Analgesia for Bone pain

• Allopurinol to prevent urate nephropathy

– Chemotherapy

– Radiotherapy

– Marrow transplantation

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• Papillary carcinoma is the most common of the malignant thyroid tumours and accounts for 90% of the irradiation induced thyroid cancer.

• It may be multifocal and spread to regional lymph nodes.

• Origin of Tumour:- Follicular cells of Thyroid gland

Papillary Carcinoma metastatic to lymph node

• From Thyroid gland Lyphoid follicle with germinal center

It metastasize evenly

• Manifestation– Cervical lymphadenopathy– With/without thyroid enlargement

• Diagnosis-– Palpable lymph nodes– Lymph node biopsy– Isotope scanning may be required.

• Management- usually by total thyroidectomy

CharactericallyOrphan annieNuclei..

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Orphan Annie- Nuclei

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Psamomma Body

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• Lymphadenitis is the most frequent form of extra pulmonary tuberculosis usually occuring in the cervical region(“ Scrofula”)

• HIV patients always demostrate multifocal disease, systemic symptoms and either pulmonary or other organ involvement due to active tuberculosis.

• It may take upto 3 year time from initial infection for TB of Lymph nodes, GIT, bones and joints.

• Clinical Manifestation– Mostly Scrofula(Lymphadenitis of cervical lymph nodes)

followed by axillary and inguinal.– Nodes are usually painless– General observation

• Weight loss, night sweats and fever.

• Diagnosis– Tuberculin test is strongly positive in these patients.– Tissue biopsy

• Management- – 4 drug therapy– Isoniazid, Rifampin, Ethambutol, Pyrizinamide

Granuloma

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Granuloma

LPO

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Reference

• Davidson’s Principles and Practice of Medicine

• Robbins- Basic Pathology

Thanking to the entire Universe

Beautiful Aurora