Abstract
The clinical presentation of patients infected with SARS-CoV-2 is remarkably diverse. Likewise, the underlying pathophysiological mechanisms are proving complex. Disturbances in the blood coagulation system and cytokine storm, such as seen in hemophagocytic syndrome, are among the most serious ones. We present the case of a female 79-year-old patient with marked thrombocytopenia of 4 (150-450) x10^9/L occurring in the context of a confirmed SARS-CoV-2 infection. Clinically, one episode of epistaxis and petechiae was observed, otherwise no signs of bleeding occurred. Diagnostic workup included microscopic blood smear analysis, bone marrow cytologic evaluation and flow cytometric immunophenotyping. Hematological malignancies, thrombotic microangiopathies and common infections were excluded as cause of the low platelet count. In the bone marrow, cytology, the megakaryocytic lineage presented normocellular. However, several large hemophagocytes with engulfed hematopoietic cells were detected. A further evaluation of markers frequently associated with hemophagocytic syndrome was performed. Ferritin was 2888 (0-150) ng/mL, CRP and GOT were slightly elevated. The white blood count was normal with a marked decrease of lymphocytes to 0.13 (1.10-3.60) × 10^9/L. There was no fever or organomegaly and the patient was in good clinical constitution. Thus, we did not diagnose hemophagocytic syndrome. Due to no other explanation for the clinical and laboratory findings, the patient was diagnosed with immune thrombocytopenia and concomitant bone marrow hemophagocytosis associated to SARS-CoV-2. The first-line treatment consisting of prednisolone and intravenous immunoglobulins failed to induce an increase in the platelet count. As second-line treatment therapy with Eltrombopag, a TPO-agonist, was started and a sustainable response with platelets in the normal range was achieved.
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