Case of 60-year-old male patient with hemophagocytosis with viral infection, a rare manifestation in sickle cell disease.
This article describes a rare case of hemophagocytosis in sickle cell disease with viral infection in a 60-year-old male patient. The patient presented to the hematology clinic with complaints of fatigue and dyspnea on exertion. The patient’s medical history revealed sickle cell anaemia with hemoglobin genotype SS.
Physical examination was remarkable of conjunctival pallor and mild scleral icterus. Further laboratory studies showed a decreased level of haemoglobin and an undetectable haemoglobin S. In addition, ferritin levels were elevated.
After admitting the patient to the hospital, doctors evaluated him for infectious causes of anaemia, including tests for parvovirus, Epstein-Barr virus and cytomegalovirus. However, results for all tests were negative. Bone marrow biopsy of the patient was remarkable of normocellular bone marrow with marked erythroid hyperplasia and hemosiderosis. Similarly, light microscopy showed numerous hemophagocytic histiocytes containing sickle cells. The findings ruled out the diagnosis of hemophagocytic lymphohistiocytosis. The test results pointed to the diagnosis of hemophagocytosis in sickle cell disease.
Hemophagocytosis
Hemophagocytosis associated with viral infections is a rare manifestation. Similarly, it is also described as a reactive process associated with blood product transfusion and bacterial sepsis. The disease causing severe anaemia is also highly unusual.
Doctors treated the patient with red-cell transfusion and iron chelation. Moreover, he continues to receive maintenance red-cell transfusion every 3 weeks.
References
Hemophagocytosis of Sickle Cells https://www.nejm.org/doi/full/10.1056/NEJMicm2021493
