Splenic infarction, splenic sequestration, and functional hyposplenism in hemoglobin S‐C disease

Abstract

Splenic atrophy or evidence of hyposplenism occurs in as many as one third of all patients with S-C hemoglobinopathy. Yet there are few reports in the literature of clinically apparent splenic infarction in this disease. We describe four instances of acute splenic infarction in three patients with hemoglobin S-C disease which illustrate a wide spectrum of clinical manifestations and severity. Of particular interest were the observations of coincident occurrences of splenic sequestration and functional hyposplenism with splenic infarction, suggesting a close pathophysiological relationship among these syndromes.

Massive Splenic Infarct in a Collegiate Football Player With Hemoglobin SC Disease

“…4 occurred in association with high altitudes and air travel, 5,6 but also without inciting events. 7 A review 8 of 14 cases of splenic infaction in HbSC disease found that patients generally presented with left upper quadrant pain (93.8%), as well as vomiting (50%), and fever (50%). Left upper quadrant tenderness was common on physical exam, with an occasional friction rub present.…”

Palpable splenomegaly in children with haemoglobin SC disease: Haematological and clinical manifestations

“…Acute splenic complications, including splenic sequestration and splenic infarction events, have been previously described in up to 38% of older patients with Hb SC ( Hook et al , 1958 ; Orringer et al , 1991 ; Sears et al , 1985 ). In children, the frequency of these events ranges from 3–6% of patients depending upon the clinical criteria, patient ages, and duration of follow‐up ( Aquino, Norvell & Buchanan .…”

“…These acute splenic sequestration events are associated with a rapidly enlarging spleen and result in anaemia, thrombocytopenia, and occasionally hypovolemic shock ( Aquino, Norvell & Buchanan, 1997; Geola, Kukreja & Schade, 1978; Orringer et al , 1991 ; Michel, Hernandez & Buchanan, 1992). Splenic infarction results from occlusion of splenic vessels; it can occur spontaneously or after a precipitating event such as exposure to high altitude ( Aquino, Norvell & Buchanan, 1997; Orringer et al , 1991 ; Sears & Udden, 1985). Clinical characteristics of splenic infarction include sudden onset of mild to moderate splenic enlargement, left upper quadrant pain, and wedge‐shaped defects on liver spleen scan ( Aquino, Norvell & Buchanan, 1997; Orringer et al , 1991 ).…”

Pleural Effusions Due to Diseases Below the Diaphragm

“…Acute splenic infarction can occur in unexpected settings, such as heterozygous sickle trait in whites at high altitudes 55 or as an intra-abdominal crisis in adult hemoglobin sickle cell disease even at sea level. 56…”