Background Familial Mediterranean Fever (FMF) is an inherited autosomal recessive disorder, caused by MEditerraneanFeVer gene (MEFV) mutations. Colchicine therapy leads to remission of the symptoms in the majority of the FMF patients. Some hematological side effects, particulary leukopenia has been reported with the use of colchicine. On the other hand, a few recent reports suggested that leukopenia apart from a side effect of colchicine treatment may be caused by FMF itself.
Objectives To evaluate hematological features of children with FMF at the time of diagnosis and during the treatment period.
Methods The medical records of patients with FMF were reviewed retrospectively. The following variables were evaluated: demographic characteristics, dosage of colchicine, and the presence of attacks despite colchicine, mutation analysis, hematological parameters (leukocyte, neutrophil, lymphocyte, monocytes, hemoglobin and platelet counts) at the time of diagnosis, and in the first and third month. Children were divided into two groups according to their age: group I<7 years, group II≥7 years.
Results A total of 51 children, 25 girls (49.0%), with a mean age of 7.47±9.87 years at diagnosis and disease duration of 13.12±9.87 months, were included. Thirteen (25.5%) patients had parental consanguinity and 18 (35.3%) patients had family history of FMF. Seven (13.7%) patients were homozygous, 18 (35.3%) patients were compound heterozygous and 18 (35.3%) patients were heterozygous for MEFV gene. Eight (15.7%) patients had complex allele mutations (≥3 mutations). Colchicine treatment was started in all patients. The dosage of colchicine needed to control the disease symptoms was 0.95±0.31 mg/day. Comparison of the leukocyte, neutrophil, lymphocyte, monocyte and platelet counts at the time of diagnosis and in the first and third months of controls, revealed no significant differences (p>0.05 for all). But, mean hemoglobin level at time of diagnosis was significantly lower than the value in the third month of colchicine treatment (p=0.001). Hemoglobin levels of Group I were significantly lower than the group II; on the other hand lymphocyte count were significantly higher in the Group I at time of diagnosis (p=0.03, p=0.02, respectively). In group I, lymphocyte counts were higher than the third month of colchicine treatment compared to time of diagnosis (p=0.01).We did not observe leukopenia in any of the patients but one patient was found to have neutropenia (neutrophil count: 1200x103/μL) at the time of diagnosis.
Conclusions Hemoglobin levels were found to be increased in all FMF patients during colchicine treatment, which might be due to the chronic inflammatory characteristics of the disease. Colchicine treatment seems to have favourable effects on hematological parameters, particularly in young children (<7 years). Prospective studies with longer follow-up times are needed for better evaluation of hematologic changes.
Disclosure of Interest None declared