High-dose methylprednisolone therapy in pure red cell aplasia.
OBJECTIVE
To report our experience using high-dose methylprednisolone (HDMP) treatment in a patient with primary acquired pure red cell aplasia (PRCA) who failed to respond to conventional prednisone therapy.


METHODS
A 29-year-old woman reported weakness, was easily fatigued, and had developed palpitations. On physical examination, pallor and splenomegaly were detected. On blood smear, mild macrocytic anemia was seen. Bone marrow aspiration and biopsy revealed normocellularity, erythroid hypoplasia (E/M: 1/10), reduction in erythroid precursors, and normal megakaryocytes and myeloid series. No disease associated with secondary PRCA was detected. Oral prednisone 1 mg/kg (total 60 mg/d) was started as conventional treatment. However, the patient's status deteriorated and the hemoglobin concentration fell from 6.5 to 5.5 g/dL within the first week of hospitalization. HDMP was then begun. Treatment protocol consisted of methylprednisolone 30 mg/kg for 4 days, 20 mg/kg for 3 days, 10 mg/kg for 3 days, 5 mg/kg for 4 days, and 1 mg/kg for 2 weeks. The patient's hemoglobin concentration increased from 5.5 to 14.2 g/dL over a period of 9 weeks. Transient hyperglycemia and cushingoid appearance were seen during prednisone treatment.


CONCLUSIONS
Exactly how steroids enhance erythropoiesis in PRCA is unknown. It seems likely that steroids render abnormal erythroid progenitors more sensitive to marrow growth factors, thereby permitting them to differentiate to functional precursors. HDMP treatment had been rarely used in patients with primary acquired PRCA. Limited studies using HDMP have shown variable results.


CONCLUSIONS
HDMP treatment may be considered safe and effective in patients with primary acquired PRCA who do not respond to conventional steroid therapy.
Kadikoylu
Gurhan
G
Department of Internal Medicine, Division of Haematology-Oncology, Adnan Menderes University Medical School, Aydin, Turkey. gurhan@medscape.com
Bolaman
Zahit
Z
Barutca
Sabri
S
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D001853:Bone Marrow / Q000473:pathology; D004906:Erythrocyte Count; D005260:Female; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D007958:Leukocyte Count; D008775:Methylprednisolone / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D010976:Platelet Count; D011241:Prednisone / Q000627:therapeutic use; D012010:Red-Cell Aplasia, Pure / Q000188:drug therapy* / Q000473:pathology
D000893:Anti-Inflammatory Agents; D006454:Hemoglobins; D011241:Prednisone; D008775:Methylprednisolone