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Splenomegaly

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Splenomegaly

  1. 1. MASSIVE SPLENOMEGALY Arphan Azaad
  2. 2. Chief Complain Distention of abdomen Left Upper Quadrant Pain(+) x 2 months Sweating+ Anorexia+
  3. 3. On Examination General Condition :Fair Vitals: Chest=B/L clear BP=100/60mmHg HR=88/min T= 36.6C R/R=20/min CVS=S1.S2.M0 P/A= Soft Massive Splenomegaly LUQTenderness+
  4. 4. ICTERIC(-) ANEMIC(-) Lymph Node(palpable nontender axillary,inguinal LN) CLUBBING(-) OEDEMA(-) DEHYDRATION(-)
  5. 5. INVESTIGATIONS COMPLETE BLOOD COUNT: WBC:16.1X10*9/L(N=20.7%),L(73.7%), RBC:3.71X10*12/L,HB:107g/L,MCV:86.8fl PLT:97X10*9/L, RET%:1.63% RENAL FUNCTIONTEST: Urea:8.9mmol/L,Cr:71umol/L Na:141mmol/L,K:4.3mmol/L
  6. 6. LIVER FUNCTIONTEST TBI:18.1umol/L,DBI:6umol/L,TP:64.6g/L,AST:17U/L SEROLOGY: HBeAg+, HBcAb+ ENDOSCOPY FINDINGS: Chronic Gastritis ,Oesophagitis
  7. 7. CT FINDINGS Massively enlarged and calcified Spleen (159.6 mm x 79.3 mm) Left Kidney cyst(14.2 x 6.4mm)
  8. 8. DIFFERENTIAL DIAGNOSIS NON HODGKIN’S LYMPHOMA HODGKIN’S LYMPHOMA CHRONIC MYELOID LEUKEMIA GAUCHER’S DISEASE MALARIA,KALAZAR MYELOFIBROSIS LIVER CIRRHOSIS
  9. 9. HL • Suggesting :painless LN+,Splenomegaly • Not suggesting:Cervical,Supraclavicular LN not palpable,BM:absence of metastatic cells NHL • Suggesting:painless axillary,inguinal LN+,,Splenomegaly • Not Suggesting : absence of Para Aortic LN,palpable,BM:absence of metastatic cells GAUCHER’S DISEASE • Suggesting:Massive Splenomegaly,Cytopenia • Not Suggesting:more common in age group<20 years,absence of Pathological fractures
  10. 10. MALARIA KALAZAR • Suggesting:massive Splenomegaly • Not suggesting:absence of fever virtually ruling out any infection MYELOFIBROSIS • Suggesting:Massive Splenomegaly • Non suggesting:absence of excessively proliferating blood cells CIRRHOSIS • Suggesting:HBeAg+,HBcAb • Non suggesting:absence of physical finding and radiological findings of Cirrhosis
  11. 11. INVESTIGATIONS REQUIRED DISEASE INVESTIGATION(GOLD STANDARD) HL & NHL LYMPH NODE BIOPSY IMMUNOPHENOTYPE MYELOFIBROSIS BM STUDY GAUCHER’S DISEASE MEASURE ACID BETA GLUCOSIDASE MALARIA,KALAZAR BLOOD SMEAR,ISOLATION OF LD BODIESON BM,SPLEEN SMEAR CIRRHOSIS NODULAR DEGENERATION OF LIVER NOTED ON RADIO AND HISTOPATHOLOGICAL STUDY OF LIVER CML BONE MARROW STUDY
  12. 12. SUMMARY  Patient clinical h/o is highly suggestive of NHL.  However we should also rule out other diseases as I have mentioned on previous slides  Elevation of CRP is highly suggestive of acute infection ,but absence of fever does not suggest it.  BM does not suggest of malignancy  SplenicVein thrombosis should be considered.
  13. 13. Absence of Hypersplenism  Hypersplenism is most commonly seen with splenomegaly due to hematologic disorder,portal hypertension,Felty Syndrome,Lymphoma.  Hypersplenism produces: Cytopenias Normal or Hyperplastic bone marrow Response to Splenectomy
  14. 14. Debatable Points  Why not to do Splenectomy for symptomatic relief of patient?  Why to use IV antibiotics so early if infective origin is least likely in the patient?  Is it worthy to consider Lysosomal Storage Disease and SplenicVeinThrombosis?
  15. 15. Neoplasm of Mature B Cells NAME ORIGIN GENOTYPE FEATURES BURKITT LYMPHOMA Germinal center B cell,CD10+ T(8,14).T(2,8) or (8,22) extranodal abdominal masses uncommonly present as Leukemia DLBLC Germinal center or post germinal center B Cell 30% have rearrangement of BCL6,10% contain translocation t(14,18) Rapidly growing mass ,30% extranodal aggressive FOLLICUAR LYMPHOMA Germinal center B cell,CD10,BCL2& BCL6 T(14,18) involving BCL2 gene Generalized Lymphadenopathy Marrow involvement MANTLE CELL LYMPHOMA Naïve B Cell,CYCLIN D1 & usually CD5 T(11,14),INVOLVIN G BCL1( Cyclin D1 & Ig H ) Disseminated,mod erately aggressive
  16. 16. DIAGNOSIS MANTLE CELL LYMPHOMA IVB Patient is currently being managed with supportive treatment to preventTumor Lysis Syndrome,managed with PPI & Prophylactic antibiotics. PLAN:TO START RCHOP REGIMEN
  17. 17. MANTLE CELL LYMPHOMA  Mantle cell lymphoma (MCL) is one of the rarest of the non-Hodgkin's lymphomas(NHLs), comprising about 6% of NHL cases.  MCL is a subtype of B-cell lymphoma, due to CD5 positive antigen-naive pregerminal center B-cell within the mantle zone that surrounds normal germinal center follicles. MCL cells generally over-express cyclin D1 due to a t(11:14)[2] chromosomal translocation in the DNA. More specifically, the translocation is at t(11;14)(q13;q32).[  The immunophenotype profile consists of CD5+ (in about 80%),[7] CD10-/+,It is usually CD5+ and CD10.[8] CD20+, CD23-/+ (though plus in rare cases). Generally, cyclin D1 is expressed but it may not be required
  18. 18. TREATMENT
  19. 19. ECOG SCORING  0 – Asymptomatic (Fully active, able to carry on all predisease activities without restriction)  1 – Symptomatic but completely ambulatory (Restricted in physically strenuous activity but ambulatory and able to carry out work of a light or sedentary nature. For example, light housework, office work)  2 – Symptomatic, <50% in bed during the day (Ambulatory and capable of all self care but unable to carry out any work activities. Up and about more than 50% of waking hours)  3 – Symptomatic, >50% in bed, but not bedbound (Capable of only limited self-care, confined to bed or chair 50% or more of waking hours)  4 – Bedbound (Completely disabled.Cannot carry on any self-care.Totally confined to bed or chair)  5 – Death
  20. 20. REFERENCE Oncopedia-guidelines.info  Wikipedia:Mantle Cell Lymphoma Harrison Principal of Medicine Pathology Robbins
  21. 21. THANK YOU

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