A Very Rare Case -  Hairy Cell Leukemia in Patient with Sarcoidosis.

Although the coexistence of hairy cell leukemia with sarcoidosis has been reported in a few cases in the literature, in our case the patient had been diagnosed and followed about 10 years with sarcoidosis and massive splenomegaly. It has been demonstrated that T helper 1 cells exist in organs influenced by sarcoidosis. These cells produce IL-2 and IFN-γ and induce a nonspecific inflammatory response and granuloma formation. Also these cytokines may play a role in the development of hairy cell leukemia.Key words: hairy cell leukemia -  sarcoidosis - massive splenomegaly.

Effect of sex difference on platelet aggregation using an optical method in healthy subjects.

There are many studies reporting conflicting results of sex differences on various platelet functions. The purpose of this study was to investigate whether sex differences could affect platelet aggregation results using an optical method in healthy subjects. A total of 42 subjects, 21 males and 21 females, were included in the study. Platelet aggregation was induced by adenosine diphosphate (ADP; 5 microm), collagen (2 microg/ml), and epinephrine (10 microm). Optical aggregation was performed using a turbidometric method. In all platelet aggregation tests ADP, collagen and epinephrine were studied; there was no significant difference between females and males in platelet aggregation amplitudes and slopes. As a result, sex difference does not affect platelet aggregation performed with this optical method in healthy subjects. This result supports that there is no need for sex differentiation while composing control groups in platelet aggregation studies using the optical method.

A chronic myeloid leukemia-like syndrome case with del (12) (p12) in a Li-Fraumeni syndrome family.

Li-Fraumeni syndrome is a familial cancer syndrome characterized by different tumors and hereditary p53 mutations. Here, a chronic myeloid leukemia-like syndrome case in a Li-Fraumeni syndrome family with del (12) (p12) cytogenetic abnormality was presented. A hereditary p53 mutation (pro309ser) supported the Li-Fraumeni syndrome diagnosis in this family. This syndrome was characterized by the clonal myeloproliferative accumulation in bone marrow and peripheral blood with negative bcr/abl gene rearrangement finding. The etiology of this rare syndrome is still unclear. This is the only chronic myeloid leukemia-like syndrome case reported in a Li-Fraumeni syndrome family. Del (12)(p12) was observed in leukemias except chronic myeloid leukemia-like syndrome. The deletion in chromosome 12p12 with hereditary p53 mutation should have a critical role in chronic myeloid leukemia-like syndrome etiology in our case.

The relationship between serum ferritin levels and disease activity in systemic lupus erythematosus.

OBJECTIVE: The aim of this study is to investigate the relationship between serum ferritin levels and disease activity in patients with systemic lupus erythematosus (SLE). METHODS: Serum ferritin levels of 72 SLE patients were measured. The SLE patients were subdivided into two groups according to SLE disease activity index (SLEDAI) as < or = 10 and > or = 11. The results were compared with 31 patients with rheumatoid arthritis (RA). 36 patients among 72 with SLE were evaluated before and after treatment. RESULTS: Serum levels of ferritin in SLE patients were higher than RA patients (p < 0.001). There was a significant difference in ferritin levels before and after treatment. The levels of ferritin in SLE were positively correlated with SLEDAI scores. Patients with SLEDAI scores > or = 11 had significantly higher serum ferritin levels. CONCLUSION: Serum ferritin levels may be a useful marker of disease activity in SLE patients.

The relationship between carotid atherosclerosis and platelet aggregation in elderly.

The aim of this study is to assess the relationship between the carotid wall intima media thickness (IMT) and atheroma plaques due to atherosclerosis and platelet aggregation among elderly. The first stage of the study was performed by analyzing platelet aggregation in a total of 28 elderly patients divided into two groups. The first group consisted of 14 cases with carotid atheroma plaque (Patient group I) and the second group of patients were without carotid atheroma plaque (Control group I). At the second stage of the study, the cases were regrouped according to the carotid IMT. Patients with IMT above 1 mm (Patient group II, n=10) and under 1 mm (Control group II, n=14) were compared regarding platelet aggregation. Platelet aggregation was induced in the platelet-rich plasma using 5 micro M ADP, 0.2 mg/ml collagen and 1.2 mg/ml ristocetin. Between patients with and without atheroma, no difference was noted in terms of platelet aggregation. Between platelet aggregation results of patients with intimal thickness above and under 1 mm, no significant difference was also noted. Between elderly cases with or without atherosclerosis, there was no difference with respect to platelet aggregation. Platelet aggregation measurements cannot be used as a marker of atherosclerosis in elderly population.

Role of pretransplant interferon-alpha(IFN) treatment in the outcome of stem cell transplantation (SCT) from related donors in chronic myelogenous leukemia (CML): results from three Turkish transplant centers.

Since transplantation cannot be performed immediately after the diagnosis of chronic myelogenous leukemia (CML), interferon treatment is usually required. This study aims to analyze the effects of interferon-alpha (IFN) treatment on allogeneic stem cell transplantation (SCT) outcome. A total of 106 patients aged 16-47 years and transplanted from HLA-identical sibling donors for CML in chronic phase (CP) were evaluated. In all, 48 had received IFN-alpha for a median duration of 5 months (1-18 months) until a median of 1 month prior to transplantation. Of the patients, 50 have received bone marrow transplant (BMT) whereas 56 have received peripheral blood stem cells (PBSCT) between 1991 and 1999 in three major transplant centers in Turkey. Patient characteristics in both groups were similar. More hematological responders were present in the IFN(+) patients (P=0.0001). No difference was found in engraftment kinetics. The incidences of acute or chronic graft-versus-host disease (GVHD), relapse and graft failure were similar in all patients regardless of stem cell source. Overall survival (OS) and disease-free survival (DFS) at 2 years were similar for both IFN(+) or (-) patients following SCT. With multivariate analysis, pretransplant IFN-alpha use, stem cell source, transplant year and CD34+ cell content were not found to be risk factors for OS. In conclusion, prior IFN exposure did not impair BMT or PBSCT outcome.

Survey of the use of complementary and alternative medicine among Turkish cancer patients.

OBJECTIVES: To measure the frequency of use of complementary and alternative medicine (CAM) among patients with malignancy undergoing or following conventional treatment, to determine demographic characteristics associated with the use of CAM, and to find out how benefits, if any, were perceived by patients. DESIGN: A 35-item survey questionnaire administered to 305 return patients with malignancy. SETTING: Clinical wards of the Oncology and Haematology departments of Gülhane Military Medical Academy, Ankara, Turkey. METHODS: Questionnaire-based measures of demographics, motives, expectations and effects of using CAM, and types and reported perceived benefits of CAM practised on patients with malignancy. RESULTS: The majority of return patients (n = 186, 61.0%) used at least one CAM practice; and birthplace, educational status, and family type were significant factors in such behaviour. CONCLUSIONS: Patients with malignancy born in villages, having less education and living in large families were more likely to use folk medicine. More than half of those using CAM (n = 99, 53.2%) reported as benefits the feelings of either strengthening of the body, being in good psychological condition, or the disappearance of several symptoms.

The relationship between gastric-oral Helicobacter pylori and oral hygiene in patients with vitamin B12-deficiency anemia.

OBJECTIVE: The aim of this study was to determine whether a relationship exists between gastric and oral Helicobacter pylori and oral hygiene in patients with vitamin B12 deficiency. STUDY DESIGN: One hundred eight patients with vitamin B12 deficiency who were H pylori -positive in their gastric mucosa were enrolled in the study. These patients were divided into 3 groups determined by Oral Hygiene Index (OHI) scores of good, fair, or poor. H pylori was detected in the dental plaque with camphylobacter-like organism test gels. All patients were treated with a combination regimen to eradicate H pylori. RESULTS: H pylori positivity in dental plaque was correlated with OHI scores; the positivity was 28.5%, 90.2%, or 100% in patients with good, fair, or poor OHI scores, respectively. The eradication of H pylori was associated with recovery from anemia and increased serum vitamin B12 level (P <.0001 and P <.0001). The patients with poor OHI scores had the most frequent gastric recurrence of H pylori (58.3%) compared with those with fair OHI scores (41.2%) and good OHI scores (4.8%). CONCLUSIONS: H pylori seems to be an etiologic factor in vitamin B12 deficiency, since anemia was cured and the level of vitamin B12 in the serum increased as a result of its eradication. However, eradication of H pylori from gastric mucosa alone is not enough to prevent gastric recurrence of the bacteria. Proper oral hygiene must be established to eliminate H pylori in dental plaque. Therefore, we suggest that control of H pylori in dental plaque is necessary to control recurrence of H pylori.

Successful treatment of severe aplastic anemia associated with human parvovirus B19 and Epstein-Barr virus in a healthy subject with allo-BMT.

Several reports have noted pancytopenia associated with Human parvovirus B19 (PVB19) or Ebstein-Barr virus (EBV) infections in patients who have no history of immunodeficiency. To our knowledge, we report the first case of severe aplastic anemia associated with both EBV and PVB19 infections in a previously healthy 22-year-old man. He was admitted to our hematology service due to anemia and thrombocytopenia. He had no symptoms or signs of infections of these viruses. His bone marrow biopsy revealed a hypocellular marrow. Specific IgM and IgG antibodies to EBV and PVB19 were elevated. EBV and PVB19 virus genomes were detected by PCR in the bone marrow nucleated cells and the peripheral blood lymphocytes. Two months after treatment with prednisone, acyclovir, and intravenous immune globulin (IVIg), the genomes of both these viruses disappeared. However, his transfusion requirement for platelet suspensions and packed red blood cells persisted. The patient underwent allogeneic bone marrow transplant (allo-BMT) and has had an enduring complete hematological response for 8 months.

Erythema annulare centrifugum as the presenting sign of CD 30 positive anaplastic large cell lymphoma--association with disease activity.

Erythema annulare centrifugum is a figurate erythema that has been associated with many different entities. A case of erythema annulare centrifugum related to non-Hodgkin's lymphoma in a 38-year-old woman is described in this case report. Response of the lymphoma to a combination chemotherapy was accompanied by disappearance of skin lesions. When therapy was discontinued, both disorders recurred, and both responded to reinstitution of a different chemotherapy regimen. To our knowledge, this case is the first reported association of erythema annulare centrifugum and non-Hodgkin's lymphoma in the medical literature.