Reverse sequestration in a case of sickle crisis

Abstract

SummaryA patient with homozygous sickle cell disease presented with sickle crisis complicated by hepatic and pulmonary sequestration and required intensive therapy. During the recovery phrase she developed a rapid rise of haemoglobin concentration, unrelated to blood transfusion, followed by hypertension, congestive cardiac failure and catastrophic intracerebral haemorrhage. This serious haemodynamic disturbance was considered to be caused by a reversal of the sequestration process. Careful monitoring ofa sickle cell patient's blood pressure, blood counts, haematocrits, haemoglobin S level and plasma viscocity, even after the end of a sequestration crisis, is recommended.

Arterial Blood Pressure and Hyperviscosity in Sickle Cell Disease

“…These authors calculated the whole blood viscosity for sickle cells at a hematocrit of 40% as equivalent to normal blood at a hematocrit of 70%. Another case was reported by Lee and Chu [42] in which transfusion was given for hepatic sequestration. Four days after the last transfusion, there was an abrupt rise in hemoglobin from 5 g/dL to 12.4 g/dL associated with abrupt onset of hypertension, congestive heart failure, and cerebral hemorrhage.…”

Unusual causes of abdominal pain: sickle cell anemia

“…36 The diagnosis of hepatic sequestration should be considered in patients with SCD with a rapid decrease in hemoglobin level and evidence of rapidly increasing hepatomegaly in the absence of an obvious cause of blood loss. Treatment includes prompt restoration of red cell mass with measures to reverse sickling process.…”

Simple chronic transfusion therapy, a crucial therapeutic option for sickle cell disease, improves but does not normalize blood rheology: What should be our goals for transfusion therapy?

“…For transfusion during an acute crisis, raising hemoglobin slowly by 3 g/dl or slowly returning to baseline hemoglobin/hematocrit is preferred. Transfusion to hemoglobin/hematocrit levels above the patient’s usual baseline, in an acute setting, may lead to hyperviscosity syndrome and worsening of the crisis event [40, 42]. The long-term goal for chronic transfusion therapy is hemoglobin of 10 g/dl and <30% HbS cells.…”