Indications:1 patient with eosinophilia-associated systemic mastocytosis.
Patients:One 30-year-old male patient. The patient was followed up for at least 19 months.
TypeofStudy:This letter to the editor presented a study that described the morphologic and immunophenotypic normalization of bone marrow mast cells in a patient with eosinophilia-associated systemic mastocytosis following treatment with Glivec. Case report.
FreeText:The patient presented with fatigue, fever, night sweat, 20-kg weight loss, splenomegaly, anemia and leukocytosis (with eosinophilia). Systemic mastocytosis with associated eosinophilia was diagnosed. The bone marrow (BM) was markedly hypercellular with aggregates of atypical mast cell infiltrates associated with intense eosinophilia, monocytosis and left-shifted granulopoiesis. Tests: BM aspiration and biopsy, stool and blood examination, electrocardiogram, echocardiogram, leukocyte (including neutrophil and eosinophil) and platelet counts, immunohistochemical staining (CD 117), flow cytometry (CD 117 and CD25 expression), BCR-ABL detection (BM cytogenetics and fluorescence in situ hybridization), and polymerase chain reaction. Other treatments: transfusion and hydroxyurea.
DosageDuration:Starting dose was 400 mg daily. After 1 week, dose was reduced to 100 mg daily. Duration not stated.
Results:Within 2 days following the initiation of Glivec therapy, the leukocyte count decreased from 69.8 × 109 to 9.4 × 109/l and the platelet count remained stable at 31 × 109/l. After 5 clays, the leukocyte count was 3.2 × 109/l with an absolute neutrophil count of 1670, an absolute eosinophil count (AEC) of below 0.1 × 109/l, and a stable platelet count. Within 4, 5 and 6 weeks, following Glivec dose reduction, the platelet, neutrophil and hemoglobin normalized, respectively, and the patient achieved complete clinical remission. Bone marrow biopsy after 3 months of Glivec therapy revealed normal histology and a marked reduction in the previously dominant aberrant CD25-positive mast cell population; the AEC was below 0.2 × 109/l. At last follow-up (19 months), the patient remains in complete remission and laboratory studies on archival tissue confirmed the presence of the FIP1-PDGFRA oncogenic mutation.
AdverseEffects:No adverse events were mentioned.
AuthorsConclusions:The current communication is the first to describe immunophenotypic normalization of bone marrow mast cells in patients with systemic mastocytosis treated with imatinib mesylate. The current case demonstrates the value of bone marrow histology enhanced with tryptase or CD117-based immunohistochemistry as well as bone marrow mast cell immunophenotyping in the monitoring of effective therapy in systemic mastocytosis.
ASJC Scopus subject areas
- Cancer Research