Acute splenic sequestration crisis (ASSC) in an adult patient with β-thalassemia sickle cell disease: a life-threatening complication

Abstract

Dear Editor, We present a case of an adult patient with sickle cell βthalassemia and a severe episode of recurrent acute splenic sequestration crisis (ASSC). A 21-year-old white man with sickle cell β-thalassemia was admitted to our hospital with headache and abdominal pain. At the age of six, he had an acute thrombotic cerebrovascular event, and since then, he was placed on repeated sessions of exchange transfusion. At the age of seven, he had suffered an episode of ASSC that was treated conservatively. Since then, the patient presented frequent pain crises that did not respond to hydroxyurea treatment. At admission, his blood pressure was 140/70 mmHg, body temperature was 38°C, and heart rate was 105 beats/min. He had hepatomegaly and splenomegaly (4 cm under the left costal margin). Hemoglobin (Hb) was 9.4 g/dl, hematocrit 29.8%, MCV 75.8 fl, white blood cell count 4,500/μl, platelet count 206,000/μl, LDH 372 U/l, and total bilirubin 2.21 mg/dl/ indirect 0.78mg/dl. HbSwas 71%. An abdominal ultrasound at admission showed an enlarged spleen (19 cm). Intravenous fluids and pain relief medications were started and the patient initially improved. On the third hospital day, the patient was suddenly found tachycardic (125 beats/min), confused, short of breath with significant hypoxemia (SatO2=80%). His Hb and platelet count dropped to 3.5 g/dl and 52,000/μl, respectively, while his LDH (1,368 U/l) and total bilirubin (3.70 mg/dl) were increased. A urinalysis was negative for Hb. A repeated abdominal ultrasound revealed an increase in the size of spleen (21 cm) with new peripheral hypoechoic areas. Intravenous fluids, oxygen, and emergency transfusion with four packed RBC units and fresh frozen plasma (FFP) were promptly administered. The patient rapidly improved and a progressive rise in Hb and SatO2 was documented. An abdominal computed tomography scan confirmed the U/S findings (see Fig. 1). The patient was transfused with two more RBC units and was discharged on the tenth hospital day with a Hb of 9.2 g/dl. An elective splenectomy was scheduled. ASSC represents a rare complication of sickle cell disease and its variants with increased mortality during the first decade of life [1–6, 14, 15]. This adult patient with sickle cell β-thalassemia presented with an acute, life-threatening, and recurrent episode of ASSC. The diagnosis was established by the acute splenic enlargement, the severe and rapid drop of Hb, and the accompanying new onset thrombocytopenia. Because his Hb decreased to 3 g/dl, this episode is considered as major by definition [8]. A minor episode is defined as a drop in Hb levels but with an Hb level >6 g/dl. Ten cases of ASSC in adults with sickle cell βthalassemia have been reported in the English literature [7–10, 14]. Of these, nine were men (90%) with a mean age of 36 years. Three of these episodes could be classified as major. The lowest Hb level recorded was 3.5 g/dl, and it was reported in our case. The mortality rate in these patients was high (50%). Four of the five (80%) surviving patients experienced recurrent ASSC [7–10, 14]. Ann Hematol (2008) 87:499–500 DOI 10.1007/s00277-007-0422-3