ABSTRACT
INTRODUCTION: Our purpose was to determine the bleeding risk of obligate and potential carriers, highlight the prophylactic applications before interventions for families and physicians. METHOD: Forty-six sisters who had at least one family member with hemophilia A or B were included. Laboratory parameters were tested.Bleeding tendency interrogated by a detailed questionnaire.The results were compared with 43 healthy female controls. RESULTS: Mean factor activity levels were significantly lower in sisters than control subjects (p = 0,004). Bleeding score was higher in sisters than controls (p = 0.001). Prolonged bleeding after minor injury was significantly higher in the sisters than control subjects (p = 0.008). Requiring further treatment due to prolonged bleeding after tooth extraction was significantly higher in sisters (p = 0.001). Sisters had postpartum hemorrhage lasting longer than 6 weeks than controls (p = 0.025). Menstrual period lasted longer in the sisters than controls (p < 0.001). Spontaneous epistaxis, oral and gingival bleeding were more frequently observed in sisters whose factor activity levels were 60 % or below (p = 0.014 and p = 0.047, respectively). There was no statistically significant difference between the severity of hemophilia in the affected family member and the factor levels in the sisters (p = 0.398).Spontaneous epistaxis has found to be significantly associated with the hemophilia severity in the family (p = 0.004). CONCLUSION: Clotting factor levels were found to be lower in the sisters and associated with spontaneous epistaxis, oral and gingival bleeding.Also, regardless of clotting factor levels, sisters significantly experienced more bleeding problems.Our study demonstrated the importance of taking precautions for prolonged bleeding in cases where medical interventions are inevitable in these patients.
Subject(s)
Hemophilia A/complications , Hemophilia B/complications , Hemorrhage/diagnosis , Adult , Female , Humans , Laboratories , Siblings , Young AdultABSTRACT
We hereby report our multicentre, retrospective experience with CLARA in patients with fludarabine/cytarabine/G-CSF (FLAG) refractory AML. The study included all consecutive R/R AML patients, who received CLARA salvage during October 2010-October 2015 period. All patients were unresponsive to FLAG salvage chemotherapy regimen and did not undergo previous allo-HCT. A total of 40 patients were included. Following CLARA 5 (12.5%) patients experienced induction mortality and 10 (25%) patients achieved CR. 25 (62.5%) patients were unresponsive to CLARA. 7 (17.5%) out of 10 patients in CR received allo-HCT. Median overall survival of patients who achieved CR after CLARA was 24.5 months (8.5-54.5) and 3 months (2.5-5), in patients who underwent and didn't allo-HCT, respectively. Our results indicate that CLARA may be good alternative even in FLAG refractory AML patients and can be used as a bridge to allo-HCT, who have a suitable donor and able to tolerate the procedure.
Subject(s)
Adenine Nucleotides/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Arabinonucleosides/administration & dosage , Cytarabine/administration & dosage , Leukemia, Myeloid, Acute/drug therapy , Neoplasm Recurrence, Local/drug therapy , Salvage Therapy/methods , Adenine Nucleotides/adverse effects , Adolescent , Adult , Aged , Arabinonucleosides/adverse effects , Clofarabine , Cytarabine/adverse effects , Drug Resistance, Neoplasm/drug effects , Female , Granulocyte Colony-Stimulating Factor , Humans , Male , Middle Aged , Retrospective Studies , Vidarabine/analogs & derivatives , Young AdultABSTRACT
Tuberculosis is still one of the most prevalent and fatal infectious diseases in spite of considerable improvements in medical science. Splenic tuberculosis is a rare form of extrapulmonary tuberculosis. There are limited numbers of cases in which immune thrombocytopenia is associated with splenic tuberculosis. We report a case of immune thrombocytopenic purpura due to splenic tuberculosis. Our case was a 58-year-old female with headache, gum bleeding, redness in legs, and ecchymoses on the arms for 10 days. On admission to hospital, laboratory tests were as follows: platelet count 6.000/mmc (150 000-450 000), haemoglobin: 12 g/dl, WBC: 8000/mm3, erythrocyte sedimentation rate: 58 mm/h and C-reactive protein was in normal ranges. After standard laboratory tests, the patient was diagnosed with idiopathic thrombocytopenic purpura. The patient presented abdominal lymphadenopathies and spleen in normal size in radiological examinations. Diagnostic laparotomy and splenectomy and lymph node excision was performed and splenic tuberculosis was detected in pathologic and microbiologic examination. The patient was successfully treated with apheresis platelets suspension, intravenous immunoglobulin and antituberculous therapy. In conclusion, splenic tuberculosis should be suspected in patients who have fever, abdominal lymphadenopathies and immune thrombocytopenic purpura. Histopathological examination is still an ideal method to confirm the diagnosis, suitably aided by microbiological examination.
