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[Hereditary spherocytosis first diagnosed upon the development of aplastic crisis; a case report]
Insights
Hereditary spherocytosis (HS) in a child was diagnosed during an aplastic crisis. Human parvovirus (HPV) infection triggered this crisis, leading to severe anemia and HS diagnosis.
Area of Science:
- Pediatrics
- Hematology
- Infectious Diseases
Background:
- Hereditary spherocytosis (HS) is a genetic hemolytic anemia.
- Aplastic crisis can exacerbate underlying hematological conditions.
- Human parvovirus B19 (HPV) is a known cause of aplastic crisis in various anemias.
Observation:
- A 6-year-old boy presented with fever and severe anemia, initially lacking jaundice and splenomegaly.
- Diagnosis of HS was confirmed by peripheral smear, osmotic fragility, and auto-hemolysis tests.
- The patient exhibited elevated IgM and IgG titers against HPV, indicating recent infection.
Findings:
- The severe anemia and reduced erythroid precursors in bone marrow suggested an aplastic crisis.
- The clinical presentation and diagnostic tests confirmed hereditary spherocytosis.
- The findings strongly link human parvovirus (HPV) infection to the aplastic crisis in this HS patient.
Implications:
- This case highlights the importance of considering viral triggers, particularly HPV, in childhood aplastic crises.
- Early diagnosis of HS is crucial, even when presenting with acute complications like aplastic crisis.
- Understanding the interplay between HS and viral infections can guide management and prognosis in pediatric hematology.
Abstract:
We report a Childhood case of hereditary spherocytosis (HS) first diagnosed upon the development of aplastic crisis. A 6-year-old boy presented with fever and anemia. Although there was neither icterus nor splenomegaly at first, mild icterus and splenomegaly gradually developed with improvement of anemia. The diagnosis of HS was made on the basis of the presence of numerous spherocytes on the peripheral smear, increased osmotic fragility and the auto-hemolysis test result. The severe anemia in the early course with a marked decrease in the bone marrow erythroid cells and the absence of icterus and splenomegaly indicate that it was due to aplastic crisis. In the virological study, anti-human parvovirus (HPV) antibody titers were increased: the values of anti-HPV IgM were high and those of anti-HPV IgG were suddenly elevated. We thus considered that this HS case developed aplastic crisis by HPV infection.