PROF.DR.P.VIJAYARAGHAVAN’S UNIT,M7 DR.K.SENTHAMIZH SELVAN

Mr.Elumalai 40 years ,male Farmer Thiruthani admitted with complaints of - fever for past 4 months - diffuse skin lesions for the past 2 months

• H/o fever – 4 months ;high grade;intermittent

; not ass. Chills /rigor;• H/o diffuse skin lesions -2 months;over extremities & trunk ;itching +; non remitting ;• H/o easy fatiguability;• H/o loss of appetite;loss of weight;• No h/o cough with expectoration ;altered bowel habits;

No h/o oral ulcers ,joint pain/swelling, discoloration of extremities ,stiffness; No history s/o jaundice ; No h/o abdominal distension,leg swelling,

oliguria; No h/o breathlessness; No h/o bleeding manifestations per orifice ; No h/o seizures /altered sensorium /LOC; No h/o dysuria /hematuria;

Not a known case of DM/SHT/IHD/BA/EPILEPSY/

No h/o contact with TB ; No h/o ATT intake ; No h/o sexual promiscuity; No h/o blood transfusions ; No h/o any surgeries ;

Personal history; Mixed diet ; Not a smoker /alcoholic;

Family history; married;two children ;no history of any familial malignancy

Conscious Oriented Febrile;103 deg.F Pallor +; Muddy conjunctiva; Generalised lymphadenopathy + ; cervical /axillary/inguinal/ discrete /firm /rubbery/nontender/ 2 ×2 cms; Diffuse hyperkeratotic nodular lesions

over the extremities & trunk

CVS – S 1, S 2 + no murmur ;

RS- NVBS + ; No added sounds ;

P/a –soft hepatomegaly -2 cm below RCM in

the MCL Splenomegaly – 4 cm below LCM in

the MCL CNS-no FND;

1. LYMPHOMA WITH SKIN CHANGES -HODGKIN’S -CUTANEOUS T CELL

LYMPHOMA

2. IMMUNOCOMPROMISED STATE -? RETRO VIRAL DISEASE

Inj.ceftriaxone 1 g iv bd

Anti pyretics

Anti histaminics IVF

Tepid sponging

13.05.2010 20.05.2010 27.05.2010

HB- 8 G/DL 7.7 G /DL 7.0 G /DL

TC -42,600 35, 700 45,000

DC – P 68% L 30% E 2 %

P 70 % L 22 % E 8% P 60 % L 28 % E 12%

RBC -3 MILLION 2.5 MILLION 2.8 MILLION

PLT- 77 000 63 000 80 000

PCV -35 % 32% 30%

ESR -64 MM HR 58 MM HR 62 MM HR

RBS -106 mg /dl Urea -24 mg /dl Creatinine – 0.8 mg /dl LFT Bi. total -1.8 mg /dl direct-0.8 mg/dl ALT- 118 U/l SGOT-80 U/L SGPT -56 U /L ALB.-3.5 G/DL GLO.-3.0 G/DL

PS –normocytic hypochromic RBC s; shift to left ; eosinophilia; thrombocytopenia;

QBC for MP – negative; MSAT –negative ; Widal –negative ; chest x ray: urine C/S –no growth ; upper mediasti Blood C/S –no growth ; -nal widening HIV ELISA –non reactive ; VDRL –negative ; LDH – 938 IU/l

USG ABDOMEN : hepatosplenomegaly ; multiple enlarged nodes in the porta hepatis ,splenic hilum; para-aortic nodes +;

FNAC OF AXILLARY NODES: Polymorphous cell population ,with lymphocytes ,plasma cells ,eosinophils , and RS like cells ;

DERMATOLOGIST OPINION: Clinically lesion suggestive of scabies advised Tab.Ivermectin Permethrin lotion HAEMATOLOGIST OPINION : Suggestive of lymphoma To do bone marrow study & node excision biopsy

lymph node parenchyma with effacement of architecture & diffuse infiltration of lymphocytes ,eosinophils ,histiocytes mononuclear and multi lobated cells

suggestive of HODGKIN’S LYMPHOMA MIXED CELLULARITY to be confirmed by immuno histo chemistry

section shows epidermis exhibiting hyper keratosis and irregular acanthosis ,dense collection of neutrophils ,lymphocytes ,and eosinophils in papillary dermis

suggestive of nodular scabies no evidence of cutaneous lymphoma

HODGKIN’S LYMPHOMA: --- MIXED CELLULARITY ---STAGE 4 B

NODULAR SCABIES :

Malignancy of lymphatic system ; 1832- Thomas Hodgkin defined the

disease ;

CLASSIFICATION 1. NODULAR SCLEROSIS 2. MIXED CELLULARITY 3. LYMPHOCYTE PREDOMINANT 4. LYMPHOCYTE DEPLETED 5.NODULAR LYMPHOCYTE PREDOMINANT

In classic HD ,neoplastic cell is RS cell ,; 1 – 2 % ; derived from the germinal centre B cell ,;rest of the cell population is lymphocytes ,plasma cells ,neutrophils, eosinophils and histiocytes;

CD marker – CD 30- Lymphoid cell - CD 15 –

granulocyte ,monocyte activated T cells ,

1-2 % of cases RS cells may be of T cell origin

60 – 80 %;

Nodular pattern; bands of fibrosis divides node into nodules Lacunar type RS cells ;mono or multi

lobated nucleus ,small nucleolus Young adults Mediastinum and supra diaphragmatic

involvement

15 – 30 % ;

RS cells classic;

large bilobate or multi lobate nucleus;eosinophilic nucleolus ;

Abdominal lymphnode ,spleen involved ;

Old age ;

Most common in HIV ;

Less than 1 % ;

Diffuse hypo cellular infiltrate ;

Large no. of RS cells and sarcomatous variants ;

EBV protein expressed ;

Old age ;

HIV

RS cells classic ,lacunar type ;

Background of lymphocytic infiltrates ;

Nodular pattern + / _;

Presentation and survival similar to mixed cellularity HD

RS cells low or absent; L + H cells /popcorn cells in the background of Lymphocytic cells; CD – 19 ,20 + 15 ,30 _

Male predominance; Bimodal distribution; Asymptomatic lymphadenopathy –above

diaphragm > 80%; Wt.loss,fever,night sweats –B symptoms-

40%; Fever –intermittent / pel ebstein’s Chest pain ,cough ,breathlessness, -mediastinal involvement Pruritus Alcohol induced pain at nodes -10 % ; Back pain /bone pain ;

Lymphadenopathy;

Splenomegaly ,hepatomegaly;

SVC obstruction ;

Paraneopalstic syndromes ; ---cerebellar degeneration; ---GBS ; ---Multi focal leukoencephalopathy; ---peripheral neuropathy;

Hyper production of cytokines by the EBV infected T cells lead to activation of the macrophages which phagocytose cells of haemoptopoietic lineage

-- pancytopenia , fever , HS megaly ,LFT abnormalities ,raised serum ferritin raised TGL ;

Infection – EBV ; 100 % in HIV ; 60 – 70 % Mixed Cellularity HD ;

15-30 % nodular sclerosis HD ;

Prolonged use of human growth hormone ;

Family history – 1 % ;

HLA –DP alleles are more common in HD ;

Raised ESR ; CBC –cytopenias ; Serum creatinine –associated with nephrotic /nephritic syndrome LDH –correlates with disease burden ; ALP Hypercalcemia /hyper natremia /hypoglycemia ; HIV /ELISA ; IL -6, IL-10,IL-2 R assay; Imaging –CT chest,abdomen ,pelvis ,PET scan; MRI – FNAC /Excision biopsy/marrow biopsy Staging laparotomy

• CT as a technique for evaluating intrathoracic and infradiaphragmatic LN

• criteria for clinical involvement of the spleen and liver modified to include evidence of focal defects with two imaging techniques & abnormalities of liver function be ignored;

• suffix 'X' for bulky disease (greater than 10 cm maximum dimension)

• a new category of response to therapy, unconfirmed/uncertain complete remission (CR[u]), be introduced to accommodate the difficulty of persistent radiological abnormalities of uncertain significance.

Stage 3 ,4 or Stage 1 and 2 with following -- ESR > 50 mm -- >3 sites -- B symptoms -- extranodal disease --bulky disease

Serum albumin < 4 g /dl ; Hb < 10.5 g /dl Male sex Stage 4 disease Age > 45 WBC > 15 000 Lymphocytes < 600 or < 8 % of total WBC 0 – 1 ---- 90 % Survival ; > 4 ---- 60 % survival ;

Treatment : Supportive care Chemotherapy /Radiotherapy; Goals of therapy ; to achieve complete remission –

disappearance of all evidence of disease ( clinical ,PET , CT ,marrow )

partial remission regression of measurable disease & no new sites of disease

Induction --MOPP --ABVD --STANFORD V --BEACOPP Salvage ( failed induction/relapse ) --ICE --DHAP --EPOCH --ESHAP High dose chemo with BMT

Monoclonal antibodies ;

SGN – 30 Targets CD 30 cells MDX- 60

For early low risk disease (1 A ,2 A ) --4 cycles of ABVD -- 8 weeks of STANFORD V foll. by XRT

Early disease with unfavourable factors ( bulky disease ) -- 6 cycles of ABVD --12 weeks of STANFORD V foll. by XRT

Advanced high risk disease (1 B ,2 B ,3 , 4 ) --8 cycles of ABVD -- BEACOPP

Local excision X RT Rituximab

If transforms into DLBCL ---R CHOP regimen Rituximab,cyclophosphamide doxorubicin ,vincristine ,prednisolone

ABVD regimen BEACOPP regimen with HAART

Most of the patients relapse in first three years

follow up every 2 – 4 months in the first year

Every 6 months for next 5 years --- CBC, LDH , ESR , TFT ,(every time ) ---X RAY CHEST ,CT CHEST ,PET Scan ( every 6 months ) ---mammography annually --- vaccines –pneumococcal ,influenza & meningococcal ---screening for secondary malignancies ---psychiatric counselling

THANK YOU