Massive erythrophagocytosis by peripheral monocytes and neutrophils in parvovirus-B19 autoimmune hemolytic anemia

L Puigví, T Baumann, S Fernández, P Castro… - Annals of …, 2017 - Springer
L Puigví, T Baumann, S Fernández, P Castro, A Pereira, A Merino
Annals of hematology, 2017Springer
Dear Editor, In response to the interesting case of diffuse large B cell lymphoma (DLBCL)
and hemophagocytosis in peripheral blood (PB) reported in [1] associated to a fatal
outcome, we would like to point out the relevance to detect this rare phenomenon and the
clinical utility for this morphologic finding. A 37-year-old male, with clinical history of severe
combined immunodeficiency (SCID), Evans syndrome, splenectomy, and autoimmune
hemolytic anemia, was admitted to the hospital because of rapidly progressive fatigue …
Dear Editor, In response to the interesting case of diffuse large B cell lymphoma (DLBCL) and hemophagocytosis in peripheral blood (PB) reported in [1] associated to a fatal outcome, we would like to point out the relevance to detect this rare phenomenon and the clinical utility for this morphologic finding. A 37-year-old male, with clinical history of severe combined immunodeficiency (SCID), Evans syndrome, splenectomy, and autoimmune hemolytic anemia, was admitted to the hospital because of rapidly progressive fatigue, jaundice, and dark urine. The SCID was diagnosed after repeated respiratory infections during infancy; it was associated to hypogammaglobulinemia and he received replacement therapy with intravenous immunoglobulin (IvIg). The first determination of Ig in our center in 1996 (18 years old) showed IgA< 0.25 g/l, IgM< 0.54 g/l, and IgG 5.9 g/l values. On physical examination, he showed pallor and no lymphadenopathy was palpable. His full blood count showed hemoglobin (Hb) concentration 63 g/l, leucocytes (WBC) 67× 109/l, monocytes 4.7× 109/l, neutrophils 39.5× 109/l, platelets 440× 109/l, and absolute reticulocyte count 100× 109/l. During several years, he showed a markedly elevated WBC and it was interpreted as a leukemoid reaction in a splenectomized patient in the context of acute infections. Differential count in the current episode was neutrophils 60%, lymphocytes 33%, and monocytes 7%. Inversion on the CD4/CD8 ratio was found without any signs of clonality (IGHV FR1 polyclonal, TCR polyclonal). Biochemical tests showed increased LDH 1562 IU/l (normal< 450), total bilirubin 15.6 mg/dl (normal< 1.2), being indirect bilirubin 13.5 mg/dl (normal< 0.6), and haptoglobin value was below the detection limit. Direct Coombs test was positive for IgG and negative for C3b/C3d. A specific real-time polymerase chain reaction for Parvovirus B19 was performed obtaining a viral load of 10,310 copies/ml. Automated PB examination using the CellaVision DM96 (Lund, Sweden) revealed massive erythrophagocytosis by monocytes and neutrophils. Images acquired on an Olympus BX43 microscope at× 1000 magnification are shown in Fig. 1.
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