FOLLICULAR LYMPHOMA- rare case presentation in hiv
- 1. A RARE CASE OF HIV ASSOCIATED LYMPHOMA UNIT – M6 CHIEF – DR. P. RAJAMAHENDRAN M.D., DCH ASSISTNT PROFESSOR – DR.VIVEK RAJA M.D., DR. MAHESHWARI M.D.,
- 2. CASE HISTORY • A 42 years old male patient admitted with C/O abdominal distention x 2 weeks, insidious onset, involving all 4 quadrants • H/o bilateral lower limb swelling, initially around ankle then progressed upto middle 1/3rd of leg • H/o fever (on & off) + • No H/o decreased urine output • No H/o facial puffiness • No H/o chest pain • No H/o cough with expectoration
- 3. • PAST HISTORY • K/C/O Old PTB, treatment completed, 2 years back • K/C/O PLWHA on ART x 3 years • Not a K/C/OT2DM/SHTN/BA/ Seizure disorder • PERSONAL HISTORY • Known chronic alcoholic & smoker
- 4. • O/E • Conscious • Oriented • Afebrile • No pallor • Not icteric • No cyanosis, no clubbing • BL pitting pedal edema • Generalised lymphadenopathy
- 5. • S/E • CVS – S1S2 +,no murmur • RS – B/L air entry+, no added sounds • P/A – Soft, distended, franks full, shifting dullness + • CNS – Conscious, moves all 4 limbs
- 6. • EXAMINATION OF LYMPH NODES • Multiple painless lymph nodes of varying sizes over B/L axillary region & B/L inguinal region, hard in consistency, fixed to skin • Skin over the nodes appear tense with dilated subcutaneous veins
- 7. 03/05 07/05 10/05 14/05 Hb 8.6 9.0 8.7 9.1 Tc 9100 9800 8300 8900 Plt 2.9 2.3 2.7 2.1 Urea 30 27 39 42 Creatinine 1.2 1.2 1.3 1.2 TB 0.3 1.1 0.7 0.9 Protein 7.3 7.1 7.7 7.1 Albumin 3.3 3.3 3.9 3.4 SGOT 17 17 17 32 SGPT 10 12 10 20 Na 139 142 141 138 K 4.1 3.9 4.2 3.7
- 8. • Sr. LDH -50 U/L • Ascitic fluid analysis • Protein 4.9 • Albumin 2.7 • LDH 936 • Ascitic fluid cytology • Smear studied shows few lymphocytes and many degenerated cells in a background of necrosis and fluid
- 10. USG ABDOMEN & PELVIS • Liver -12cm • Gall bladder - Distented • Spleen - 13cm • Right kidney - 9.8 x 3.5 cm, increased echoes, CMD+, • Left kidney – 10.2 x 3.5 cm , increased echoes, CMD+, BL HUN+ • Urinary bladder – Distended • Prostate- normal • FF in abdomen and pelvis • Pancreas – multiple cyst seen • Suggested CT abdomen
- 11. MGE OPINION • ADVICE • Ascitic fluid ADA/ CBNAAT • Sputum C & S, Sputum AFB • CECT abdomen to R/O Peritoneal TB
- 12. CTM OPINION • IMP - PTB relapse/ PLWHA / HIV Associated lymphoma • Advice • To start ATT • To continue ART drugs
- 17. • FNAC Inguinal node • Smear showed high cellularity composed of dispersed predominantly monomorphus population of intermediate to large cell with prominent nucleoli in a hemorrhagic background • IMPRESSION – Suggestive of NON HODGKIN’S LYMPHOMA • Advised lymph node excision biopsy for further evaluation
- 19. DIAGNOSIS • HIV associated Lymphoma ( Follicular lymphoma) • PTB relapse on ATT • PLWHA on ART
- 20. TREATMENT GIVEN • DIL • Vitals monitoring • Inj. 25 % D IVTDS • IVF 2 unit NS @ 50ml/hr • Inj. Piperazallin tazobactum 4.5gms IV TDS • Inj.Thiamine 200mg IV OD • ART – TLD regimen
- 21. DISCUSSION • Follicular lymhoma is the second most frequent subtype of nodal lymphoid malignancies in western Europe • The annual incidence of this disease has rapidly increased during the recent decades • It is a neoplasm of follicle center B cell that is composed of mixture of centrocytes and centroblasts
- 22. ETIOLOGY • Genetics – chromosomal translocation • Viruses – EBV, HTLT I, HHV 8 • Immunodeficiency state : • Congential • Acquired – HIV , Immunosuppresant drugs
- 23. PATHOGENESIS • Follicular lymphoma is thought to arise from germinal center B cells • The pathogenesis of FL is incompletely understood • Approximately 85% of FL have t(14; 18), an oncogene that blocks apoptosis, leading to prolonged cell survival
- 26. CLINICAL PRESENTATION • New painless lymphadenopathy • Non tender, firm, rubbery in consisitency • Mostly involves retroauricular, cervical, supraclavicular, axillary, inguinal, popliteal rarely epitrochlear nodes • Extranodal sites includes spleen, liver, bone marrow • B symptoms – Fever > 38, night sweats, weight loss • Anemia, leukopenia, thrombocytopenia due to BM suppression
- 27. GRADING • WHO morphological grading • Grade I - < 5 centroblasts/ HPF • Grade II – 6 to 15 centroblasts/HPF • Grade III - > 15 centroblasts/HPF • IIIa centrocyte still present • IIIb fully contains centroblasts
- 29. LIMITED STAGE DISEASE STAGE I : Involvement of a SINGLE lymph node region OR IE: Involvement of a SINGLE EXTRALYPMHATIC site/organ STAGE II: Involvement of TWO or more lymph node regions on the SAME SIDE of the diaphragm OR IIE: Involvement of ONE lymph region and one CONTIGUOUS EXTRALYMPHATIC organ/site on the SAME side of the diaphragm
- 30. ADVANCED STAGE DISEASE STAGE III : Involvement of lymph node regions on BOTH SIDES of the diaphragm OR IIIS: Involvement of lymph node regions ABOVE the diaphragm along with involvement of the SPLEEN STAGE IV : Diffuse/ disseminated involvement of one or more extralypmhatic organs/site with/without nodal involvement OR NON CONTIGUOUS EXTRALYMPHATIC ORGAN involvement with nodal STAGE II disease OR Any extralymphatic involvement with nodal STAGE III Involvement of any of these – CSF, bone marrow, liver , lungs
- 31. Follicular Lymphoma International prognostic Index- FLIPI ADVERSE FACTORS Age > 60yrs No.of nodal sites involved >4 LDH>upper limit of normal Ann Arbor Stage III-IV Heamoglobin <12gm/dl No.of Factors Risk Group 5-year OS(%) 10-yr OS(%) Prognosis 0-1 Low 91 71 Good 2 Intermediate 78 51 Moderate 3-5 High 52 36 Poor
- 32. TREATMENT • Treatment of FL is not curative, except in early stage disease • Since treatment is not curative, treat onlyif there are indications of treatment
- 33. GELF CRITERIA • High tumor bulk defined by either • A tumor > 7cm • 3 nodes in 3 distinct areas each >3cm • Symptomatic spleenic enlargement • Organ compression • Ascities or pleural effusion • Presence of systemic symptoms • Serum LDHG or ß2 macroglobulin above normal values
- 34. BNLI CRITERIA • Rapid disease progression the preceeding 3 months • Life threatening organ involvement • Renal or liver infiltration • Bone lesions • Systemic symptoms or pruritic • Hb< 10gm/dl orWBC < 3000/mm3 or platelet count < 1 lakh/ mm3 , related to marroe involvement
- 35. CHEMO I MUNOTHERAPHY • I – 3a : BR • 3b : R-CHOP • Maintanence with RITUXIMAB once in 3 months for 2 years
- 36. TREATMENTS IFRT (30-35 Gy) Stage I,II 22- 33% Watch and Wait R- CHOP Stage III,IV 67- 78% Follicular Lymphoma (grade I,II) R-CHOP + IFRT Stage I,II Watch and Wait R- CHOP Stage III,IV Follicular Lymphoma (grade III) = curable = incurable Indolent Aggressive
- 37. TREATMENT • Waxing waning course- wait and watch low grade (I, II) • Most radio sensitive • Radiotherapy uesd for localised disease • Involved field or extended field RT ranging from 35 to 50Gy • Achieve remission in 50% patients
- 38. • Single agent chemotherapy • Chlorambucil • Fludarabine • Rituximab • Idelasilib –phophoinositide 3 kinase delta inhibitor ( Pl3k)
- 39. • Multi agent chemotherapy • R-CVP • R – CHOP • FCR • RFCM • FR
- 40. THANKYOU