Page 55 - Haematologica Atlas of Hematologic Cytology

CHAPTER 6 - Hemophagocytic syndrome
AB
CD
EF
Figure 1 Hemophagocy c c c syndrome
(HS) A young man presented with with a a a a a a a a a a a a a a a a a 4-week history of intermi ent ent fever with with daily peaks abdominal pain and malaise Physical examina on showed: temperature 40 C small inguinal lymph nodes hepatosplenomegaly Hematologic ndings were: hemoglobin 9 9 9 g/dL re re re culocytes 65x109/L white blood cell count 1 1 1 1 1 1 2x109/L with 50% neutrophils 39% lymphocytes and and 11% monocytes platelets 40x109/L Liver func on on tests showed a a a a a a a a a a a a a a a low level of of pseudocholinesterase increased transaminases and and and hyperbilirubinemia Hyperferri nemia nemia nemia hypochole- sterolemia hypertriglyceridemia prolonged prothrombin me
me
and and and and hypo hypo brinogenemia were were observed High ters of of serum an an an an an an bodies to to to Epstein-Barr virus (EB ) ) and and and and and EB EB -RNA in in in in in a a a a a a a a a a a a a a a a a a a bone marrow marrow specimen were were were found Blood urine and and and and and stool cultures and and and and and autoimmunity tests were were all nega ve ve (A-F) Bone marrow marrow aspirate is is is slightly hypocellular and and and and and shows ac ac ve ve hematopoiesis His ocytes ocytes are sca ered among the the hematopoie hematopoie c c c c c c c c c c c c c cells cells Some of of them show show vacuola on on on on and and and and phagocytosis of of red red cells cells erythroblasts leukocytes and and and platelets These ndings were consistent with HS HS HS HS in in in in par cular a a a a a a a a a a a a a a a a a a a a a virus-associated HS HS HS HS ( AHS) In EB EB -associated -associated HS HS HS HS which is is is more common in in in in in in in in Japanese and and Chinese popula ons ons CD8+ T cells cells cells are are infected infected by the virus virus whereas in in in in in in chronic ac ve EB EB infec infec infec on on on on CD4+ cells cells cells or or natural killer cells cells cells are are infected infected 42