Multi-organ failure associated with acute parvovirus infection and exercise in a patient with sickle beta thalassemia.

To The Editor: Multi-organ failure is a rare and life-threatening complication of sickle cell anemia and sickle thalassemia. (1-3) To our knowledge, this is the fourth reported case of multi-organ failure associated with sickle thalassemia. (1) Patients present with abrupt, precipitous drops in hemoglobin and platelet counts, fever, and respiratory and liver failure.

A 26-year-old female with history of sickle beta thalassemia and smoking presented with one day of severe pleuritic chest pain and dyspnea following exercise. No prior complications from sickle thalassemia were reported. The patient's examination revealed scattered rales and wheezes. Laboratory evaluation revealed hematocrit (33%), mean corpuscular volume 77.6 (fl), creatinine (1.7 mg/dL), normal cardiac enzymes, and normal ventilation perfusion scan.

The patient was admitted. Intravenous morphine and hydration were initiated. On day two of admission, the patient abruptly became hypoxic, hypotensive, and febrile. Hematocrit dropped to 25.8% and platelets to 56,000/m[m.sup.3]. The patient also developed acute pancreatitis and liver failure with moderate coagulopathy. Peripheral smear revealed abundant sickled cells but no schistocytes. Hemoglobin electrophoresis demonstrated 68.6% hemoglobin S, 19.6% hemoglobin A1, 4.8% hemoglobin A2, and 7% hemoglobin F. Parvovirus IgM antibodies were positive, and blood cultures were negative. Spiral chest computed tomographic scan was negative for pulmonary embolus and demonstrated new right lobe infiltrate. Echocardiogram was normal. Rapid blood transfusion, fresh frozen plasma, vasopressor support, mechanical ventilation, and antibiotics were initiated. After one week, she was extubated and weaned off pressors. Repeat hemoglobin electrophoresis revealed a hemoglobin S drop to 20%. She was discharged home stable after two weeks with resolution of all laboratory abnormalities, and was counseled on smoking cessation and the risks of exercise.

Acute multiple organ failure associated with sickle cell anemia or sickle thalassemia is both a rare and potentially catastrophic complication. Precipitating factors in our patient were exercise, acute parvovirus infection, and smoking. Other known precipitants include dehydration, acidosis, and hypoxemia, which promote sickling of cells. (1,2) Hassel (2) described seventeen cases of sickle cell anemia associated with multi-organ failure, defined as severe organ dysfunction of two of the three following organ systems (respiratory, hepatic, or adrenal systems). Vaso-occlusive events are postulated as the underlying pathogenesis in sickle cell anemia. Alpha and beta thalassemia proteins ameliorate clinical complications. Rapid reversal of multiple organ failure with simple and/or exchange transfusion support the theory of microvascular occlusion by sickled cells. (1-3)

Sickle chest syndrome and respiratory failure is postulated to arise from fat emboli from infarcted bone, pneumoniae, atelectasis, and micro-emboli. (3) Liver failure is theorized to occur from plugging of hepatic sinusoids by bile and sickle red blood cells, leading to vascular stasis and local hypoxia. (1) Acute pancreatitis is felt secondary to biliary obstruction or microvessel occlusion and ischemic injury. (4) Renal failure is attributable to hypovolemia or acute tubular necrosis from microthrombosis of the renal vasculature by sickle cells. Therapy is supportive and addressed at the underlying sickling, including hydration, oxygenation, pain control, antibiotics, and simple or exchange transfusion. (1,2,4) There was no mortality in these four cases of multiorgan failure and sickle thalassemia, and two of the four cases, including this case, did not require exchange transfusion.

References

1. Tedla FM, Friedman EA. Multi-organ failure during a sickle cell crisis in sickle/beta thalassemia. Am J Kidney Dis 2003;42:1-4.

2. Hassel KL. Acute multi-organ failure syndrome: a potentially catastrophic complication of severe sickle cell pain episodes. Am J Med 1994;96:155-162.

3. Salzman S. Does splinting from thoracic bone ischemia and infarction contribute to the acute chest syndrome in sickle cell disease? Chest 2004;122:1-5.

4. Ahmed S, Siddiqui AK, Siddiqui RK, Kimpo M, Russo L, Mattana J. Acute pancreatitis during sickle cell vaso-occlusive painful crisis. Am J Hematol 2003;73:190-193.

Hien Nguyen, MD

Connie Le, MD

Department of Medicine

George Washington University

Washington, DC