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Biomedical subjects

B Zülfikar

Publications and source records attributed to B Zülfikar.

7 recordsLinked to original sources
PubMed2002

Two patients with haemophilia and acute leukaemia.

Acute leukaemia is the commonest form of malignancy in childhood. The coincidental development of leukaemia in children or adults with haemophilia is extremely rare, although cases of leukaemia and other malignancies have been reported previously in HIV-positive subjects. Of a total of 440 people with haemophilia registered with our society, two were diagnosed with acute leukaemia last year. The development of leukaemia in a subject with haemophilia has previously been reported from our country in 1985, but the negative HIV status of these recent cases is very interesting. The first case involved a 14-year-old boy with moderate haemophilia A, who developed acute lymphoblastic leukaemia (ALL) [French-American-British (FAB) classification L2]. The second subject was a 16-year-old boy who had moderately severe haemophilia A with no previous family history, and developed acute nonlymphocytic (myelomonocytic) leukaemia (FAB-M4). Both patients received conventional chemotherapy and this report discusses the potential problems in management of such cases, including diagnosis and administration of chemotherapy in subjects with a pre-existing haemorrhagic disorder. Extensive cutaneous and mucosal bleeding, as well as bleeds in joints previously affected by haemarthrosis and alterations of haematological values were all initially suggestive of the development of inhibitors against factor VIII, but the appearance of blasts in the peripheral blood and bone marrow led to the definitive diagnosis. The risk of bleeding, due to the combination of both leukaemia and the consequences of the chemotherapy, was overcome by the administration of coagulation factor concentrates (daily initially followed by prophylactic doses after successful induction of remission in both patients). The young patient with ALL is now receiving the maintenance phase of the Children's Cancer Study Group 1961 protocol and is in the 15th month of follow-up, without any complications. The other case relapsed in the seventh month, developing enterobacter sepsis, and died. An important lesson to be learnt from these cases is that the possible diagnosis of leukaemia should not be overlooked in a patient with haemophilia and severe haemorrhagic problems, if the first-line differential diagnosis of inhibitor development against factor VIII (or IX) has been excluded.

Adolescent↗
PubMed1996

Circumcision ceremonies at the Ottoman palace.

Circumcision is widely practiced in all Islamic countries. Festivities pertaining to circumcision vary according to the regions and civilizations. In this report, circumcision festivities at the Ottoman Palace and the socioeconomic importance of the tradition are presented. The Ottoman circumcision technique is discussed, as are the miniature paintings, in manuscript, written on the occasion of the circumcision of the sons of the Sultans. Because these festivities involved the participation of all classes of the society and all professions they contributed to social and technical progress and led to developments in art, music, sports, and ideas.

Circumcision, Male↗
PubMed1996

Monosomy 7 myeloproliferative disease associated with neurofibromatosis type I: a case report.

A 7-year-old girl with neurofibromatosis type I (NF1) was diagnosed to have monosomy 7 myeloproliferative disease (Mo 7-MPD). Of the benign and malignant tumors that are encountered with increased incidence in NF1, those originating from the neural crest are frequent. However, tumors that do not originate from the neural crest may also be seen and among these, myeloid leukemias are prominent. Studies on NF1 patients with Mo 7-MPD and juvenile chronic myeloid leukemia (JCML) have suggested the role of the NF1 gene in the leukemogenesis. The relationship between monosomy 7 and hematological malignancies is already known. These findings are in agreement with the multi-step development theory of cancer. In addition, our case is one of the very rare NF1 cases having father to daughter inheritance with a myeloid malignancy. We believe that cytogenetic and molecular genetic studies will contribute to further understanding of leukemogenesis.

Child↗
PubMed1992

Ewing's sarcoma: experience with 12 cases.

Twelve patients with localized Ewing's sarcoma were treated between 1980-1990 at the Istanbul School of Medicine, Department of Pediatric Oncology-Hematology, Oncology Research and Treatment Center and Our Children Leukemia Foundation. There were 8 boys and 4 girls, with a mean age of 8.1 (range 3-17) years. The tumors were in the femur in 3 patients, in the humerus and rib in 2 patients each and in the tibia, radius, vertebra, clavicula and pelvis in 1 patient each. Chemotherapy alone was applied in 2 patients, 1 patient had chemotherapy and radiotherapy. The remaining 9 cases were treated with Chemotherapy and radiotherapy (during the chemotherapy). The chemotherapy protocols were: VAC (n = 5), VACA (n = 3), IVAD (n = 3) and T.9 (n = 1). One patient died from the disease itself. Remissions were achieved in the other 11 patients. After 5 to 95 months (mean: 22 months) 7 patients had relapsed (4 had local and 3 had distant metastases). Three patients were not able to be followed, 3 died due to additional problems (infection, cardiotoxicity). The best prognosis was achieved when Ewing's sarcoma initiated in the long bones, with less than 100 ml tumor volumes and patients were under 5 years old. There were no significant differences among chemotherapy protocols.

Adolescent↗
PubMed1991

The efficacy of mezlocillin-amikacin combination in febrile neutropenic children with oncologic disease.

The efficacy of mexlocillin-amikacin combination as empirical therapy for febrile neutropenic patients was studied in 30 children (21 males, 9 females) with various oncologic diseases aged 1-15 years (mean age 7.3 +/- 4.4) in the Istanbul Medical School, Oncologic Disease Research and Treatment Center, and Department of Pediatric Hematology-Oncology between January 1 and May 31, 1988. The response rate was 76.6%. Profound persistent granulocytopenia (fewer than 100 ml) was present in 70% of the patients. In 63.3% of patients, the infections were microbiologically documented (60%) Gram(+) and 40% Gram(-). The combination was well tolerated with hepatic and/or renal disturbances in 8 cases (26.6%). We conclude that mezlocillin-amikacin is an effective empirical combination in the initial treatment of infections in febrile neutropenic children with various oncologic diseases.

Adolescent↗