a case of hodgkin's disease
TRANSCRIPT
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PROF.DR.P.VIJAYARAGHAVAN’S UNIT,M7 DR.K.SENTHAMIZH SELVAN
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Mr.Elumalai 40 years ,male Farmer Thiruthani admitted with complaints of - fever for past 4 months - diffuse skin lesions for the past 2 months
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• 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;
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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;
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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 ;
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Personal history; Mixed diet ; Not a smoker /alcoholic;
Family history; married;two children ;no history of any familial malignancy
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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
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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;
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1. LYMPHOMA WITH SKIN CHANGES -HODGKIN’S -CUTANEOUS T CELL
LYMPHOMA
2. IMMUNOCOMPROMISED STATE -? RETRO VIRAL DISEASE
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Inj.ceftriaxone 1 g iv bd
Anti pyretics
Anti histaminics IVF
Tepid sponging
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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
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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
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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
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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 ;
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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
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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
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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
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HODGKIN’S LYMPHOMA: --- MIXED CELLULARITY ---STAGE 4 B
NODULAR SCABIES :
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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
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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
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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
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15 – 30 % ;
RS cells classic;
large bilobate or multi lobate nucleus;eosinophilic nucleolus ;
Abdominal lymphnode ,spleen involved ;
Old age ;
Most common in HIV ;
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Less than 1 % ;
Diffuse hypo cellular infiltrate ;
Large no. of RS cells and sarcomatous variants ;
EBV protein expressed ;
Old age ;
HIV
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RS cells classic ,lacunar type ;
Background of lymphocytic infiltrates ;
Nodular pattern + / _;
Presentation and survival similar to mixed cellularity HD
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RS cells low or absent; L + H cells /popcorn cells in the background of Lymphocytic cells; CD – 19 ,20 + 15 ,30 _
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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 ;
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Lymphadenopathy;
Splenomegaly ,hepatomegaly;
SVC obstruction ;
Paraneopalstic syndromes ; ---cerebellar degeneration; ---GBS ; ---Multi focal leukoencephalopathy; ---peripheral neuropathy;
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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 ;
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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 ;
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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
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• 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.
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Stage 3 ,4 or Stage 1 and 2 with following -- ESR > 50 mm -- >3 sites -- B symptoms -- extranodal disease --bulky disease
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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 ;
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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
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Induction --MOPP --ABVD --STANFORD V --BEACOPP Salvage ( failed induction/relapse ) --ICE --DHAP --EPOCH --ESHAP High dose chemo with BMT
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Monoclonal antibodies ;
SGN – 30 Targets CD 30 cells MDX- 60
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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
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Local excision X RT Rituximab
If transforms into DLBCL ---R CHOP regimen Rituximab,cyclophosphamide doxorubicin ,vincristine ,prednisolone
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ABVD regimen BEACOPP regimen with HAART
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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
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THANK YOU
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