hemophagocytic syndrome
DESCRIPTION
HPSTRANSCRIPT
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HEMOPHAGOCYTIC SYNDROME
Dr. Sandip Dukare
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INTRODUCTION
• Hemophagocytic syndrome is a rare disorder of the mononuclear phagocytic system.
• It is characterized by benign proliferation of mature histiocytes and uncontrolled phagocytosis of the platelet, erythrocytes, lymphocytes and their hematopoietic precursors in the bone marrow.
• It is associated with various infections,autoimmune ds, malignancies and immunocompromised states.
• Cytopenias result in systemic manifestations.
• Prognosis is grave and mortality ranges upto 50% in absence of treatment.
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CASE REPORT 1: HPS associated withPlasmodium falciparum infection
• 11-month-old child presented with 20-day H/O- high fever and abdominal distention.
• G/E-lethargic, febrile.
• P/E-pallor, hepatosplenomegaly.
• Lab investigations-anemia,thrombocytopenia, leucopenia,deranged liver enzymes.
• P/S-microcytic, hypochromic picture with no e/o any haemoparasite.
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• Serum ferritin -2193 μg/L.
• Platelet count- 90 X109/mm3.
• Serum triglycerides- 3.2 mmol/L.
• Serum fibrinogen-(0.75 g/L).
• BMA- normal cellularity with mature monohistiocytes containing phagocytosed RBCs.
• BMS- Plasmodium falciparum gametocytes were identified.
• Treatment with i.v. Artesunate,the patient became afebrile & recovered gradually.
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Hemophagocyte in bone marrow smear; (Leishman, ×100)
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Gametocyte of Plasmodium falciparum in bone marrow smear; (Leishman, ×100)
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CASE REPORT 2: HPS - A cause for fatal outcome in tuberculosis.
• A 17-year-old male presented with fever,abdominal distention since 15 days.
• G/E-gross pallor,generalized lymphadenopathy.
• USG A/P-free fluid in abdomen,hepatosplenomegaly and extensive intra-abdominal lymphadenopathy.
• CXR- B/L pleural effusion.
• Lab investigations-
• Anemia,leucopenia thrombocytopenia.
• Peripheral smear—microcytic,moderately hypochromic anemia.
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• Serum LDH- (540 U/L).
• Serum triglycerides- (280 mg/Dl).
• Serum ferritin- (960 mcg/L),
• CRP-positive.
• Cervical lymph node biopsy-dilated sinuses containing macrophages with abundant cytoplasm stuffed with red blood cells.
• BMA-hypocellularity, focal necrotic areas. The M/E ratio was normal. Myloid and erythroid series maturation was normal.
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• BMS- few lymphocytes, reactive plasma cells and large histiocytes containing engulfed red blood cells, nuclear debris and platelets.
• Cervical lymph node aspiration cytology- necrotic material and acid fast bacilli on ZN staining.
• The patient was started on antitubercular therapy & dexamethasone.
• Significant response was noted and patient recovered gradually.
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Histopathology of cervical lymph node. H and E (×400)-Sinus histi ocytosis with prominant hemophagocytosis
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Leishman Stain highlighting hemophagocytic macrophage
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Bone marrow aspirate (×1000) . Leishman stained smearshowing hemophagocytic macrophages
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DISCUSSION
• Hemophagocytic syndrome/hemophagocytic lymphohistiocytosis(HLH)
• It is a rare ds.
• Characterized by benign proliferation of the mature histiocyte.
• There is uncontrolled phagocytosis of the platelet, erythrocytes, lymphocytes and their hematopoietic precursors in the BM giving rise to cytopenias.
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PRIMARY(Familial)
SECONDARY(Acquired)
INFECTIONIMMUNODE-
FICIENCYAUTOIMMUNE
METABOLIC DS.
MALIGNANCY
ETIOLOGY
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PATHOPHYSIOLOGY
Th 1 STIMULATED HYPERCYTOKINEMIA
TRIGGERING OF THE CYTOKINE CASCADE
FREE OXYGEN RADICAL RELEASE
ACTIVATED MACROPHAGES PHAGOCYTOSE RBCS,WBCS,PLATELETS
INAPPROPRIATE ACTIVATION & UNCONTROLLED PROLIFERATION OF THE MACROPHAGES
TRIGGERING FACTOR (MC INFECTION)
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CLINICAL FEATURES
• Onset- abrupt• Many present with fever of unknown origin.• Systemic manifestations-pallor,fever,rash,
lymphadenopathy,hepatosplenomegaly,neurological manifestations.
• It takes a fulminant course and has a fatal outcome.
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Histiocytic Society Protocol Criteria
1. Fever(>7days)
2. Splenomegaly
3. Cytopenias(>2 lineages)
-Anemia(hb<9.0 g/dl)
-Neutropenia(<1000)
-Thrombocytopenia(<1lk cells)
4. Hypertriglyceridemia & Hypofibrinigenemia
5. Haemophagocytosis(bone, spleen,bone marrow)
6. Natural killer cell activity(low/absent)
7. Hyperferritinemia(>500 mcg/l)
8. Increased soluble CD 25(>2400 u/ml)
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Differential Diagnosis
1. Griscelli syndrome.
2. X linked lymphoproliferative syndrome.
3. Autoimmune lymphoproliferative syndromes.
4. Macrophage activation syndromes.
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REFERENCES1. Imashuku S. Differential diagnosis of hemophagocytic
syndrome:Underlying disorders and selection of the most effective treatment.Int J Hematol 1997;66:135-51.
2. Saribeyoglu ET, Anak S, Agaoglu L, Unuvar A. Secondary hemophagocyticlymphohistiocytosis induced by malaria infection in a child with langerhans cell histiocytosis. Pediatr Hematol Oncol 2004;21:267-72.
3. Ohno T, Shirasaka A, Sugiyama T, Furukawa H. Hemophagocytic syndrome induced by plasmodium falciparum malaria infection. Int J Hematol 1996;64:263-6.
4. Aouba A, Noguera ME, Clauvel JP, Quint L. Hemophagocyticsyndrome associated with plasmodium vivax infection. Br J Haematol 2000;103:832-3.
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