Pediatrician. Pediatrics Department, University Hospital of Fuenlabrada. Madrid. Spain.
Pediatrics Resident. Pediatrics Department, University Hospital of Fuenlabrada. Madrid. Spain.
Afr Health Sci. 2022 Mar;22(1):598-601. doi: 10.4314/ahs.v22i1.69.
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.
To describe the case of a patient with SCD and splenic sequestration, in which the suspicion of concomitant aplastic crisis affected her prognosis.
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.
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.
镰状细胞病（SCD）是一种遗传性血红蛋白病，其特征是红细胞中存在血红蛋白 S。这种聚合物会扭曲红细胞，使微循环阻塞，半衰期更短，导致慢性溶血性贫血。微小病毒 B19 会使这种贫血恶化，因为它会损害红系前体细胞，导致红细胞生成减少。这些患者有时会出现脾功能亢进，表现为急性血液被困在脾脏中，伴有低血容量性休克的临床症状。这两种情况同时出现会导致极其严重的情况，需要紧急行动。
描述一例镰状细胞病和脾功能亢进患者的病例，怀疑同时发生再生障碍性危象影响了她的预后。
一名 3 岁女孩患有纯合子 SCD，表现为发热、咳嗽、呕吐和下肢疼痛。入院时，观察到血流动力学不稳定、黏膜苍白和脾肿大。入院时的血象显示血红蛋白水平急性下降，网织红细胞减少。怀疑发生脾功能亢进和再生障碍性危象，因此给予输血，随后病情逐渐改善。血清中检测到人类微小病毒 B19 特异性 IgM 和 IgG 抗体。
镰状细胞病和微小病毒 B19 感染患者必须密切观察脾肿大情况，因为早期发现脾肿大可以早期诊断这种并发症。
