Parvovirus B19: A cause for aplastic crisis and hemophagocytic lymphohistiocytosis

Abstract

A 17-year-old female patient with hereditary spherocytosis (HS) presented with fatigue, nausea, headache, and fever for 2 days. On physical examination, she had high fever, pallor, icterus, and mild splenomegaly. Peripheral blood examination demonstrated a Hb level of 8.2 g/dL, WBC 1.9 Â 10 9 /L, platelets 74 Â 10 9 /L, MCV 88.9 fL, MCH 33.9 pg, MCHC 38.1 g/dL, and reticulocyte 0.7%. The peripheral blood smear revealed 70% neutrophils, 18% lymphocytes, 10% monocytes and 2% band neutrophils, vacuolization in neutrophils and monocytes; many spherocytes. Bone marrow aspirate showed hypocellularity with many hemophagocytic histiocytes, giant proerythroblasts, and absence of mature erythroblasts, suggesting a parvovirus infection (Fig. 1). On the second day of hospitalization, her Hb level decreased to 5.6 g/dL. Hepatomegaly developed and splenomegaly progressed. Serum ferritin level was 7,478 ng/mL, triglyceride 1.17 nmol/L and fibrinogen 2.31 g/L. Prothrombin and partial thromboplastin time were within normal limits, but the D-dimer level was very high (3,004 mg/L). The serum folic acid level was normal. Parvovirus B19 IgM and IgG was found to be positive by indirect immunofluoroscent assay. Secondary hemophagocytic lymphohistiocytosis (HLH) and aplastic crisis due to Parvovirus B19 infection was diagnosed. She received red blood cell transfusions and intravenous immunoglobulin was given for the HLH. She was discharged from the hospital after a marked clinical and hematological improvement on day 6 with a Hb level of 9.9 g/dL, WBC 4.8 Â 10 9 /L and platelets 198 Â 10 9 /L. Ten days later, her 13-year-old brother, who also had HS, presented with similar clinical features and Parvovirus B19 infection. He improved with red blood cell transfusions and supportive treatment. In patients with hemolytic anemia, with shortened red cell survival time and expanded marrow erythropoiesis, Parvovirus B19 infection can lead to a transient aplastic crisis. Giant proerythroblasts and absence of mature erythroblasts are characteristic findings of parvovirus infection [1][2][3].

Postrenal Transplant Hemophagocytic Lymphohistiocytosis and Thrombotic Microangiopathy Associated with Parvovirus B19 Infection

“…Since the first report of B19 associated HLH (20), few similar cases have been described (21)(22)(23)(24). To date about 30 such cases have been reported in children and adults.…”

“…Since the first report of B19 associated HLH (20), few similar cases have been described (21)(22)(23)(24) (25)(26)(27)(28). Hence, performing an extensive workup for the infectious causes of HLH is necessary as some infections can trigger HLH and the management is different depending on the case.…”

Debate around infection-dependent hemophagocytic syndrome in paediatrics

“…HPS has been reported in 28 cases of parvovirus B19 infection, most of whom had hereditary spherocytosis as the underlying disease: fewer than half were children [64][65][66][67][68]. Of these patients, 16 did not receive any treatment and 22 survived, thus suggesting that the prognosis of parvovirus-associated HPS is better than that of the other viral-mediated forms of HPS.…”

Viral infections associated with haemophagocytic syndrome

“…Approximately 30 cases [70,[133][134][135][136][137][138][139] of parvovirus B19 infection have been associated with HPS. The most common underlying disease was hereditary spherocytosis.…”