We summarize here a case involving a fatal sickle cell crisis in a patient with previously very mild hemoglobin sickle cell (Hb SC) disease. With granulocyte colony-stimulating factor (G-CSF) priming for peripheral stem cell collection, a crisis developed, followed by death within 36 hours. The case strongly suggests a role for granulocytes in acute sickle cell complications and a need for cautious use of G-CSF in this disease. The patient was a 47-year-old African American woman who 6 weeks earlier learned that she had Hb SC disease. She had no history of sickle cell crises, although she occasionally had some joint pains that always resolved within a few hours after 1 or 2 doses of aspirin. She had pneumonia as a child, but no history of strokes, lower extremity ulcerations, gallstones, splenic infarctions, hypersplenism, bone infarcts, hip necrosis, blood transfusions, or ophthalmologic problems. Hb SC disease was diagnosed after a hemoglobinopathy evaluation at the time of HLA typing, done in preparation for her to become a stem cell donor for her sister, who had chronic myeloid leukemia (CML) and mild Hb SC disease. The patient was the only sibling and had a 6 of 6 antigen match. After transplantation committee and ad hoc member reviews and careful discussions with donor and recipient, the patient proceeded with treatment to donate stem cells. She started daily G-CSF injections (400 g/M2) and dexamethasone (10 mg/M2, starting on day 3) and noted some aches and pains in her back, legs, and shoulders. But on the fourth day of treatment, she awoke with the worst pain of her life, primarily affecting her lower back and thighs. She had no associated fevers, chills, dyspnea, cough, nausea, vomiting, diarrhea, or neurologic symptoms.

The admission physical exam revealed a well-developed and wellnourished woman writhing in pain. The vital signs included a temperature of 37.0 C (98.6 F), a pulse rate of 85, a respiratory rate of 24, and blood pressure of 138/94. The eyes had no scleral icterus. Results of the lung and heart exams were normal. The abdomen was nondistended with normal bowel sounds and flexed muscles but no obvious guarding or tenderness. No masses were noted. Lumbar and sacral spine regions, posterior pelvis, and both femurs were markedly tender. The skin and neurologic exams were normal. Table 1 shows the remarkable admission studies: the white blood cell (WBC) count differential had 72% segmented neutrophils, 21% band forms, 2% metamyelocytes, 2% lymphocytes, and 3% monocytes. Intravenous hydration, scheduled morphine, and a single dose of ketorolac achieved good pain control. The next morning, the patient felt