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Acute hepatitis with prolonged fever1
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Acute hepatitis with prolonged fever1 Presentation Transcript

  • 1. Acute Hepatitis and prolonged fever – How far do we go? Presenter: Anita Dhanrajani - Jaslok Hospital, Mumbai Moderator: Ujjal Poddar – Pediatric Gastroenterologist, SGPGI, Lucknow Panelists: Gaurav Narula - Pediatric Oncologist, Tata Hospital, Mumbai Prakash Vaidya – Pediatrician, Fortis Hospital, Mumbai Geeta Billa – Hepatologist, Hiranandani Hospital, Mumbai
  • 2. Acute hepatitis with prolonged fever – how far do we go? Dr Anita Dhanrajani Under guidance of Dr Aabha Nagral Jaslok Hospital And Research Centre
  • 3. HISTORY • 17 y, M • BONCM • Presenting complaints of fever for 10 days and jaundice for 15 days. • Right upper quadrant pain • Anorexia, vomiting, malaise and fatigue • Fever low grade, intermittent
  • 4. HISTORY • No h/o drug intake • No history of blood transfusions, sexual contact • No previous history of jaundice • No history of alcohol intake
  • 5. EXAMINATION • • • • • Tachycardia Icterus Liver palpable: 4 cm, tender, firm, sharp edge Spleen palpable: 2 cm BLCM Enlarged submandibular lymph nodes: 2.5 cm
  • 6. COURSE • • • • Rapidly progressed to altered sensorium Grade 3 encephalopathy – needing ventilation Developed haematemesis and malena Abdominal distension with presence of free fluid • Fever subsided
  • 7. INVESTIGATIONS CBC Hb – 10.2gm% WBC: 4500/mm3 P 28, L70 PC – 1.1 lacs LFTs T Bili – 13.8 ALP – 40 IU SGPT – 1740 SGOT – 1028 TSP/Alb: 4.8 gm%, 1.9 gm % Viral markers IgM anti HAV – positive IgM anti HEV – Positive HBV, HCV, HIV negative USG abdomen Mild ascites, hepatomegaly, splenomegaly Coagulatio PT – 28/12 n profile INR – 2.3 Others S ammonia – 110 RBS – 44 mg% S Blood culture creatinine – - negative
  • 8. PRIMARY DIAGNOSIS Acute viral hepatitis (A+E) with fulminant hepatic failure (FHF) • Prodrome followed by jaundice and then encephalophathy • High bilirubin with high ALT • Prolonged PT • High ammonia • IgM anti HAV plus HEV positive
  • 9. FURTHER COURSE • Encephalopathy recovered – patient extubated • Fever reappeared – high grade • Tachycardia, tachypnea • Hepatomegaly, splenomegaly and lymphadenopathy persisted • Continuing coagulopathy Any thoughts?
  • 10. Cause of fever? • • • • Peritonitis (SBP) Urinary tract infection Ventilator induced pneumonia Septicemia What next?
  • 11. FURTHER INVESTIGATIONS CULTURES BLOOD negative URINE – negative ASCITIC FLUID – negative SPUTUM negative RADIOLOGY USG – CT brain – ascites, normal hepatospleno megaly PET CT whole body – adenopathy XRAY CHEST – normal IMMUNOLO GY IgG – 1839 (N:664-1401) IgM – 385 (N:55-141) IgA – 200 (N:103-404) C3 : 64 (73135) C4 : 9.9 (1272) VIRAL MARKERS CMV – negative EBV negative INVASIVE Lymph node biopsy – reactive hyperplasia with atypical Bone marrow biopsy - ? myelodysplas ia
  • 12. What next?
  • 13. BONE MARROW REVIEWED
  • 14. Other investigations • • • • Ferritin: 18,080 ug/L [N:15-200 ug/L] Triglycerides: 642 mg/dl [N:150 mg/dl] Fibrinogen: 150 mg/dl [N: 200 – 400 mg/dl] NK cell activity and perforin levels sent – could not be assessed due to low number of lymphocytes
  • 15. FINAL DIAGNOSIS Secondary Haemophagocytic Lymphohistiocytosis (HLH) – secondary to viral hepatitis
  • 16. FURTHER COURSE AND TREATMENT • • • • • Started on treatment as per HLH 2004 protocol Dexamethasone - 10mg/m2 daily Etoposide - 150 mg/m2 twice weekly Cyclosporine - 6 mg/kg daily Prophylactic co trimoxazole – 5 mg/kg thrice weekly • Continued antibiotics and antifungals – Cefoperazone, Teicoplanin, Amphotericin B , Colistin
  • 17. FURTHER COURSE AND TREATMENT • Child developed severe neutropenia after 3 doses of etoposide (ANC <500) • Discontinued etoposide • Hypotension, hypoxia – required mechanical ventilation and inotropic support • Succumbed to illness
  • 18. When to suspect HLH CLINICAL CLUES Unremitting , undiagnosed prolonged fever LABORATORY CLUES Rapidly progressive cytopenias Organomegaly – splenomegaly, lymphadenopathy Hyperferritinemia Hypertriglyceridemia Hypofibrinogenemia Bleeding manifestations BM or Organ biopsy with hemophagocytosis
  • 19. DIAGNOSIS OF HLH • The diagnosis of HLH can be established if one of either 1 or 2 below is fulfilled: 1. A molecular diagnosis consistent with HLH is made. 2. Diagnostic criteria for HLH are fulfilled (5 of the 8 criteria) • Fever • Splenomegaly • Cytopenias (affecting 2-3 lineages in the peripheral blood):hemoglobin 9 g/dL, platelets 1 lac, neutrophils 10 • Hypertriglyceridemia and/or hypofibrinogenemia: fasting triglycerides 3.0 mmol/L (ie, 265 mg/dL), fibrinogen 1.5 g/L • Hemophagocytosis in BM, spleen, or lymph nodes • Low or absent NK-cell activity.
  • 20. TAKE HOME MESSAGE • Secondary HLH high index of suspicion – can complicate any viral illness. • Investigations for primary HLH should form part of initial work up in any given HLH case • Initial treatment of primary and secondary HLH – same.