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CASE SCENERIO
BY
Dr. HUSSNAIN QADIR
PGR
WARD-19
The 1 ½ year old girl brought to OPD with C/O
increasing pallor and bleeding gums from the past 2
days. Mother gives H/O fever for the last 1 ½ month for
which she has been treated symptomatically with no
relief.
O/E she is markedly pale, febrile with temp 101FO
and pulse of 140/min there is some ooze of blood from
the gums and few purpuric spots on trunk. Spleen is
palpable 6cm below costal margin and liver is enlarged
2cm below costal margin. Her investigations show TLC
2.9x109/L, platelet 40000/mm3 and Hb of 5gm/dl.
Her serum ferritin levels are 3000 ng/dl and she is
having Tevel of 300 mg/dl.
a) What is the most probable diagnosis ?
b) Name 2 other investigations to confirm diagnosis ?
c) Enlist diagnostic criteria ?
d) Name drugs used for treatment ?
a) MAS (secondary hemophagocytic sundrome or
hemophagocytic lymphohistiocytosis)
b)
1.ESR (decreased)
2. bone marrow aspiration (macrophage
hemophagocytosis)
Diagnostic Criteria
• Laboratory Criteria
• Cytopenia
• Abnormal liver function tests
• Coagulopathy (hypofibrinogenemia)
• Decreased ESR
• Hypertriglyceridemia
• Hyponatremia
• Hypoalbuminemia
• Hyperferritinemia
• Elevated CD25 and CD163
• Clinical Criteria
• Nonremitting Fever
• Hepatomegaly
• Splenomegaly
• Lymphadenopathy
• Hemorrhages
• Histopathologic Criteria
• Macrophage hemophagocytosis in the bone marrow aspirate
• Increased CD163 staining of the bone marrow
d)
1. High Dose intravenous
methylprednisone
2. Cyclosporine
3. Interleukin-1 inhibitors
i. Anakinra,
ii. Canakinumab
4. interleukin-6 receptor antagonist
Tocilizumab
THANK YOU

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Secondary haemophagocytic syndrome

  • 1. CASE SCENERIO BY Dr. HUSSNAIN QADIR PGR WARD-19
  • 2. The 1 ½ year old girl brought to OPD with C/O increasing pallor and bleeding gums from the past 2 days. Mother gives H/O fever for the last 1 ½ month for which she has been treated symptomatically with no relief. O/E she is markedly pale, febrile with temp 101FO and pulse of 140/min there is some ooze of blood from the gums and few purpuric spots on trunk. Spleen is palpable 6cm below costal margin and liver is enlarged 2cm below costal margin. Her investigations show TLC 2.9x109/L, platelet 40000/mm3 and Hb of 5gm/dl. Her serum ferritin levels are 3000 ng/dl and she is having Tevel of 300 mg/dl.
  • 3. a) What is the most probable diagnosis ? b) Name 2 other investigations to confirm diagnosis ? c) Enlist diagnostic criteria ? d) Name drugs used for treatment ?
  • 4. a) MAS (secondary hemophagocytic sundrome or hemophagocytic lymphohistiocytosis)
  • 5. b) 1.ESR (decreased) 2. bone marrow aspiration (macrophage hemophagocytosis)
  • 6. Diagnostic Criteria • Laboratory Criteria • Cytopenia • Abnormal liver function tests • Coagulopathy (hypofibrinogenemia) • Decreased ESR • Hypertriglyceridemia • Hyponatremia • Hypoalbuminemia • Hyperferritinemia • Elevated CD25 and CD163 • Clinical Criteria • Nonremitting Fever • Hepatomegaly • Splenomegaly • Lymphadenopathy • Hemorrhages • Histopathologic Criteria • Macrophage hemophagocytosis in the bone marrow aspirate • Increased CD163 staining of the bone marrow
  • 7. d) 1. High Dose intravenous methylprednisone 2. Cyclosporine 3. Interleukin-1 inhibitors i. Anakinra, ii. Canakinumab 4. interleukin-6 receptor antagonist Tocilizumab