Acute human parvovirus B19 infection triggers immune‐mediated transient bone marrow failure syndrome, extreme direct hyperbilirubinaemia and acute hepatitis in patients with hereditary haemolytic anaemias: multicentre prospective pathophysiological study

Abstract

Summary A total of 244 patients with hereditary haemolytic anaemias (HHA) were screened for acute symptomatic human parvovirus B19 infection (HPV‐B19) in a prospective study. To assess the risks associated with HPV‐B19 infection, patients were classified into Group I and Group II according to presence or absence (symptoms, signs and specific serology) of acute HPV‐B19 infection respectively. In all, 131 (53·7%) patients had β‐thalassaemia, 75 (30·7%) hereditary spherocytosis (HS), 27 (11·1%) sickle cell anaemia (SCA) and 11 (4·5%) glucose‐6‐phosphate dehydrogenase (G6PD) deficiency. Of 33 (13·5%) patients who presented with symptomatic HPV‐B19 infection, 19 (57·5%) had HS, nine (27·3%) had β‐thalassaemia and five (15·2%) had SCA. In Group I, there were significant differences in the mean white blood cell, red blood cell and platelet counts, haemoglobin concentration, total bilirubin (TB), alanine aminotransferase, aspartate aminotransferase and serum creatinine (all P < 0·001) compared to Group II. In all, 27 (81·8%) patients had arthropathy and bone marrow failure (BMF); 13 (39·4%) had acute kidney injury (AKI), more in SCA (80%); and 12 (36·4%) patients had hepatitis, more in HS (66·8%). Five (15·2%) patients with HS had BMF, AKI, nervous system involvement and extreme hyperbilirubinaemia (TB range 26·3–84·7 mg/dl). Five (15·2%) patients had haemophagocytic syndrome. Two patients with HS combined with Type‐I autoimmune hepatitis presented with transient BMF. Complete recovery or stabilisation was noted at 12 months in every patient except for one patient with SCA who died during the infection. HPV‐B19 must be suspected and screened in patients with HHA with typical and atypical presentations with careful follow‐up.

Autosomal Dominant Hereditary Spherocytosis In An Elderly Patient

“…Patients with HS may experience severe medical conditions due to the presence of two comorbidities, even though HS itself is not considered a life-threatening condition. The most worrisome concomitant disease in patients with HS is parvovirus B19 infection 7 .…”

Case Report: Parvovirus B19 infection complicated by hemophagocytic lymphohistiocytosis in a heart-lung transplant patient

“…It is associated with pure red cell aplasia (PRCA) ( 2 – 4 ), viral myocarditis ( 5 – 8 ), erythema infectiosum ( 9 ), and other clinical manifestations. At the same time, evidence of the presence of parvovirus B19 has also been found in bone marrow transplant recipients ( 10 ) and diseases such as systemic lupus erythematosus ( 11 , 12 ), miscarriage ( 13 ), systemic sclerosis ( 14 ), hereditary hemolytic anemias ( 15 ). Infectious erythema is one of the most common clinical manifestations of parvovirus B19 infection, which often occurs in children ( 4 ).…”

“…Viral reactivation can occur in proerythrocytes and myocardial cells, and could be the cause of multi-organ damage ( 4 – 8 ). The pathogenic effects of parvovirus appear to be immune-mediated ( 5 – 8 , 11 , 12 , 14 , 15 ). Besides, the expansion of viral inclusion bodies in proerythroblasts mediating erythroid maturation arrest has also been observed in PRCA patients suggesting the direct pathogenic effect of the virus ( 2 , 16 ).…”

Autoimmune Hemolytic Anemia as a Complication of Congenital Anemias. A Case Series and Review of the Literature

“…The chronic course of both hemoglobinopathies and CHAs may be complicated by the abrupt drop of Hb values due to several causes, including increased destruction/sequestration (i.e., hemolytic crisis) and reduced/inhibited erythropoiesis (i.e., aplastic crisis). The latter recognizes various triggers, particularly parvovirus B19 infection [ 9 ], while the former is mainly immune-mediated. In particular, alloantibodies (alloAbs) are usually seen in chronically transfused patients and may cause severe transfusion reactions.…”