摘要：Background
Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by the presence of hemoglobin S in red blood cells. This polymerizes, distorting the red blood cells, which occlude the microcirculation and have a shorter halflife, giving rise to a chronic hemolytic anemia. This anemia is worsened by parvovirus B19, as it compromises the erythroid precursor, causing a decrease in erythrocyte production. These patients sometimes present with splenic sequestration, characterized by acute blood entrapment in the spleen, with clinical signs of hypovolemic shock. The simultaneous appearance of both leads to an extremely severe situation that requires urgent action.
Objective
To describe the case of a patient with SCD and splenic sequestration, in which the suspicion of concomitant aplastic crisis affected her prognosis.
Clinical case
3-year-old girl with homozygous SCD, presenting with fever, cough, vomiting and pain in the lower limbs. Upon arrival, hemodynamic instability, mucocutaneous pallor, and splenomegaly were observed. Hemogram on admission showed an acute drop in haemoglobin level with reticulocytopenia. Splenic sequestration was suspected, along with aplastic crisis, so she received a blood transfusion, subsequently showing progressive improvement. Human parvovirus B19-specific IgM and IgG antibodies were detected in the serum.
Conclusion
Patients with SCD and parvovirus B19 infection must be closely observed for splenomegaly since an early identification of an enlarging spleen can lead to an early diagnosis of this complication.
