2. 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.
3. CASE REPORT 1: HPS associated with
Plasmodium 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 investigationsanemia,thrombocytopenia,
leucopenia,deranged liver enzymes.
• P/S-microcytic, hypochromic picture with
no e/o any haemoparasite.
4. •
•
•
•
•
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.
7. 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 intraabdominal lymphadenopathy.
• CXR- B/L pleural effusion.
• Lab investigations• Anemia,leucopenia thrombocytopenia.
• Peripheral smear—microcytic,moderately
hypochromic anemia.
8. •
•
•
•
•
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.
9. • 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.
10. Histopathology of cervical lymph node. H and E (×400)Sinus histi ocytosis with prominant hemophagocytosis
13. 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.
15. PATHOPHYSIOLOGY
TRIGGERING FACTOR
(MC INFECTION)
INAPPROPRIATE ACTIVATION &
UNCONTROLLED PROLIFERATION
OF THE MACROPHAGES
Th 1 STIMULATED
HYPERCYTOKINEMIA
TRIGGERING OF THE
CYTOKINE CASCADE
FREE OXYGEN RADICAL
RELEASE
ACTIVATED MACROPHAGES
PHAGOCYTOSE RBCS,WBCS,PLATELETS
16. 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.
19. REFERENCES
1. 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.