Trop spl syndr

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Trop spl syndr

  1. 2. Department of Medicine Dr. Haji Khan H.Khoharo Assistant Professor Department of Medicine
  2. 3. <ul><li>Mr Soomar, male of 19 years presented with complaint of </li></ul><ul><ul><ul><ul><li>fever on/off since 3-4 years </li></ul></ul></ul></ul><ul><ul><ul><ul><li>lump in abdomen-left side n below the umbilicus </li></ul></ul></ul></ul><ul><ul><ul><ul><li>abdominal fullness </li></ul></ul></ul></ul><ul><ul><ul><ul><li>loss of appetite </li></ul></ul></ul></ul><ul><ul><ul><ul><li>generalized weakness </li></ul></ul></ul></ul><ul><ul><ul><ul><li>pale skin </li></ul></ul></ul></ul><ul><ul><ul><ul><li>multiple blood transfusions </li></ul></ul></ul></ul>
  3. 4. Physical Examination <ul><li>Pulse, BP - normal </li></ul><ul><li>Anemia - +ve </li></ul><ul><li>Temperature- 102 F </li></ul><ul><li>Pale conjunctivae n skin </li></ul><ul><li>lymph node not palpable </li></ul><ul><li>Chest- clear with vesicular breathig/rhonchi </li></ul><ul><li>CVS- Normal </li></ul><ul><li>CNS- intact </li></ul><ul><li>Abdomen- revelaed massive splenomegaly and mild hepatomegaly. </li></ul>
  4. 5. LABS. <ul><li>Blood CP -revelaed pancytopenia </li></ul><ul><li>Blood MP- +ve on many occasions(P.Vivax) </li></ul><ul><li>anti HCV, HBsAg- -ve </li></ul><ul><li>LFT -normal </li></ul><ul><li>Ultrasound- massive splenomegaly n mild liver enlargement. </li></ul><ul><li>IgM-elevated </li></ul><ul><li>Urine DR </li></ul><ul><li>Blood sugar </li></ul><ul><li>Uric acid </li></ul><ul><li>Serum LDH </li></ul>
  5. 6. Differential Diagnosis <ul><li>Tropical Splenomegaly Syndrome </li></ul><ul><li>Leukemia </li></ul><ul><li>Lymphoma </li></ul><ul><li>Myeloproliferative disorders </li></ul><ul><li>Kala-azar </li></ul><ul><li>Leishmaniasis </li></ul>
  6. 7. DISCUSSION <ul><li>A diagnosis of “Tropical Splenomegaly syndrome(TSS)” was made. </li></ul><ul><li>TSS(Hyper-reactive malarial splenomegaly) </li></ul><ul><li>Diagnostic criteria: </li></ul><ul><li>1.massive splenomegaly </li></ul><ul><li>2.hepatomegaly </li></ul><ul><li>3.Ig M level 2X </li></ul><ul><li>4. Clinical n immunological response to long term antimalarial Rx. </li></ul>
  7. 8. PATHOGENESIS <ul><ul><ul><li>Repeated malarial infection </li></ul></ul></ul><ul><ul><ul><li>Production of IgM- antisuppresor Ig </li></ul></ul></ul><ul><ul><ul><li>Inhibition of Suppressor T cells </li></ul></ul></ul><ul><ul><ul><li>Uninhibited B-cell stimulation </li></ul></ul></ul><ul><ul><ul><li>Overproduction of IgM and cryoglobulins </li></ul></ul></ul><ul><ul><ul><li>Reticuloendothelial system overstimulation </li></ul></ul></ul><ul><ul><ul><li>Progressive enlargement of spleen n liver </li></ul></ul></ul>
  8. 9. Spleen <ul><li>Massively enlarge </li></ul><ul><li>dilated sinusoids </li></ul><ul><li>Reticuloendothelial cells </li></ul><ul><li>Marked erythro-phagocytosis </li></ul><ul><li>Leuko/ thrombo-phagocytosis </li></ul><ul><li>Lymphocytice infiltration of splenic pulp. </li></ul>
  9. 10. LIVER <ul><li>Hepatomegaly </li></ul><ul><li>Sinusoidal dilatation </li></ul><ul><li>Lymphocytic Infiltration </li></ul><ul><li>Kupffers cells - hyperplasia </li></ul><ul><li>Hyperfunctioning Reticulo-endothelial function </li></ul><ul><li>Phagocytosis of rbc, wbc, platelet </li></ul>
  10. 11. Abdominal Findings
  11. 12. Management <ul><li>Blood transfusion </li></ul><ul><li>Proguanil 200mg/day for adults </li></ul><ul><li>Chloroquine- Prolonged time period/life long </li></ul><ul><li>Pyrimthamine </li></ul><ul><li>-------following are not indicated </li></ul><ul><li>Splenectomy </li></ul><ul><li>Splenic irradiation </li></ul><ul><li>Antimitotic therapy </li></ul>
  12. 13. Prognosis <ul><li>Good/condition is reversible with antimalarial </li></ul><ul><li>Increased mortality bz of secondary infections </li></ul><ul><li>Anemic cardiac failure </li></ul><ul><li>Splenic lymphoma </li></ul>
  13. 15. Thanks

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