Acute splenic sequestration in a pregnant woman with homozygous sickle-cell anemia

Abstract

CONTEXT: Homozygous (SS) sickle-cell anemia complicated by acute splenic sequestration in adults is a rare event, and it has never been reported during pregnancy. CASE REPORT: A 25-year-old woman with homozygous (SS) sickle-cell disease was hospitalized at 32 weeks' of gestation presenting weakness, abdominal pain, fever and hemoglobin of 2.4 g/dl. Abnormal fetal heart rate was detected by means of cardiotocography, and 5 units of packed red cells were transfused. Cesarean was performed at 37 weeks. Both mother and baby were discharged in a good general condition.CONCLUSION: This case report demonstrates the importance of immediate blood transfusion for treatment of fetal distress in cases of splenic sequestration during pregnancy. This treatment is essential for avoiding maternal and fetal complications.RESUMO CONTEXTO: Anemia falciforme homozigótica (SS) complicada por sequestro esplênico agudo em adultos é evento raro, e nunca foi relatado durante a gravidez. RELATO DO CASO: Uma mulher de 25 anos, portadora de doença falciforme homozigótica (SS), com 32 semanas de gestação, foi internada apresentando fraqueza, dor abdominal, febre e hemoglobina de 2,4 g/ dl. Frequência cardíaca fetal anormal foi detectada pela cardiotocografia e a paciente recebeu 5 unidades de concentrado de hemácias. Cesariana foi realizada com 37 semanas. Mãe e filho receberam alta em bom estado geral. CONCLUSÃO: Este relato de caso demonstra a importância da transfusão imediata para o tratamento de sofrimento fetal nos casos de sequestro esplênico durante a gestação. Este tratamento é imprescindível para se evitarem complicações maternas e fetais.

Splenopathy in Patients with Sickle Cell Disease

“…Treatment of acute splenic sequestration mainly consists of early diagnosis, clinical support, and packed RBC transfusion [24]. In patients who survive an attack, subsequent splenectomy is usually performed to avoid the potentially life-threatening complications of recurrence [25,26].…”

“…First, patients with acute splenic syndrome usually undergo splenectomy if their condition can be managed conservatively and splenic rupture has occurred [14]. Second, in patients who survive an acute splenic syndrome attack, subsequent splenectomy is usually performed to avoid the potentially life-threatening complications of recurrence [25,26]. Third, patients with symptomatic splenomegaly due to SCD are candidates for splenectomy [31].…”

Adverse outcome of acute splenic sequestration crisis in pregnancy

“…3,4 There are only three published reports of ASSC in pregnancy; with a neonatal death in 1972, and no reported adverse fetal outcomes in the subsequent two cases. 5 Three further reports describe ASSC postpartum. 5,6…”

“…Sequelae of ASSC may include hypovolemic shock and death, and efforts to promptly restore circulating blood volume are paramount. 1,3 The acute decrease in oxygenation associated with an abrupt and often profound hemoglobin drop has the potential to result in fetal death or fetal brain injury due to hypoxia, 4,5 particularly if maternal hemoglobin acutely falls below 60 g/L. 5…”

“…1,3 The acute decrease in oxygenation associated with an abrupt and often profound hemoglobin drop has the potential to result in fetal death or fetal brain injury due to hypoxia, 4,5 particularly if maternal hemoglobin acutely falls below 60 g/L. 5…”