An autopsy series of an oft-missed ante-mortem diagnosis: hemophagocytic lymphohistiocytosis

Majumder, Anusree; Sen, Debraj

doi:10.4322/acr.2021.243

Cited by 2 publications

(1 citation statement)

References 28 publications

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“…16 Two recent case series showed the relationship between clinical HLH features and postmortem pathological hemophagocytosis in autopsy patients. 17,18 The previous study which compared HLH to non-HLH reported that the prevalence of hemophagocytes was 18.4 (HLH) versus 4.3 (non-HLH) per 1000 cells in bone marrow 19 In the present case, 30 hemophagocytes per 1000 cells were detected, indicative of remarkably increased hemophagocytosis. The study also reported that nonnucleated erythrophagocytosis alone is a non-specific finding, while hemophagocytosis of granulocytes, nucleated erythrocytes and lymphocytes, and hemophagocyte containing multiple nucleated cells are strongly associated with HLH.…”

Section: Discussionmentioning

confidence: 43%

Catastrophic antiphospholipid syndrome with Epstein–Barr virus-associated hemophagocytosis: A clinicopathological conference

Nawata

Shirayama

Oshida

et al. 2022

Lupus

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Catastrophic antiphospholipid syndrome (CAPS) is a severe variant of antiphospholipid syndrome associated with multiorgan thrombosis in a short term. We present the case of a 14-year-old immunocompetent girl who developed renal, intestinal, and pulmonary infarction; thrombocytopenia; and hemolytic anemia within 1 week. She was diagnosed with thrombotic microangiopathy. Hence, plasma exchange and corticosteroid therapy were initiated, which improved thrombocytopenia. However, the patient’s platelet count decreased. Her general condition gradually worsened with eventual death. An autopsy revealed multiple infarctions in the kidneys bilaterally, jejunum, ileum, and pulmonary parenchyma. Microthrombi were not detected. Massive hemophagocytosis was observed in the splenic pulp, lymph nodes, and bone marrow. Several Epstein–Barr viruses (EBVs)-encoded small ribonucleic acid (RNA)-positive lymphocytes were also found in the bone marrow. The presence of antibodies to both viral capsid antigen-immunoglobulin G and EBV nuclear antigen indicated past infection. Antiphospholipid antibody was positive after her death. The patient was finally diagnosed with CAPS and EBV-associated hemophagocytosis, possibly due to EBV reactivation. Establishing a clinical diagnosis of CAPS was relatively difficult because two different causes of thrombocytopenia, CAPS and hemophagocytosis, led to a difficulty in understanding this case’s pathogenesis.

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