Coexistence of chronic neutrophilic leukemia with multiple myeloma

A case report of simultaneous presentation of chronic neutrophilic leukemia and multiple myeloma (IgG kappa) in a 71-year-old male is described. The patient showed mature neutrophilic leukocytosis, hepatosplenomegaly, high neutrophil alkaline phosphatase score, hyperuricemia, neutrophils with toxic granulation and Dohle bodies, absence of Philadelphia chromosome and of the bcr-abl fusion gene. Moreover, a monoclonal IgG kappa paraproteinemia (36.93 g l(-1)) was detected. Bence-Jones proteinuria was 3.84 g l(-1). The bone marrow was grossly hypercellular with marked myeloid hyperplasia and aggregates of plasma cells. The patient died of severe bronchopneumonia after the transformation of chronic neutrophilic leukemia to acute myelomonocytic leukemia, 1.5 years following diagnosis

Clonality of acquired primary pure red cell aplasia: Effectiveness of antithymocyte globulin

Primary pure red cell aplasia (PRCA) was diagnosed in two male patients, 65 and 69 years old respectively. In both, surface markers of peripheral blood nuclear cells revealed the presence of TCRalphabeta+ phenotype. Clonality of T cells was confirmed by the polymerase chain reaction in both patients, in whom, prednisone at a dose of 1 mg/kg/day improved the anemia and lower doses caused its renewal, resulting in the reappearance of the patient's transfusion requirement. On the other hand, the anemia seems to have been treated permanently (second case) with horse antithymocyte globulin (ATG) (20 mg/kg/day 1 to 8 +) since his hemoglobin was about 15 g/dl at the time of writing. In the first patient, the hemoglobin level was 10.5 g/dl one month after the administration of ATG (15 mg/kg/ d 1 to 5 +), but unfortunately, the patient died because of a massive gastrointestinal bleeding on the fortieth day following this treatment. We, therefore, suggest that, patients with acquired primary PRCA should be screened to detect the presence of a T-cell clone and recommend that, treatment should start earlier with ATG, if the PRCA is due to a T-cell clonal disorder

T-cell large granular lymphocytic leukaemia: successful response to 2-deoxycoformycin

We report a 25-year-old woman with T-cell large granular lymphocytic leukaemia presenting with severe neutropenia, anaemia and recurrent infections with a chronic disease course. Immunophenotyping showed an expansion of CD3+, TCR gamma delta(+), CD4-, CD5+, CD7+, CD8+, CD57+ large granular lymphocytes. Clonality was demonstrated with T-gamma polymerase chain reaction analysis which revealed clonal rearrangement of the TCR gamma chain gene. Cyclosporine, granulocyte colony-stimulating factor, methothrexate and a combination of cyclophosphamide, vincristine and prednisolone failed to correct the neutropenia and the anaemia. Finally, treatment with 2-deoxycoformycin resulted in both clinical and haemotological complete responses, despite molecular evidence of the persistence of the abnormal T-cell clone

Automated Auditory Brainstem Response: A Proposal for an Initial Test For Healthy Newborn Hearing Screening with a Focus on the Test Time

Objective: Some researchers recommend the use of Automated Auditory Brainstem Response as an initial screening test for healthy newborns due to its lower false-positive and referral rates, high specificity, and also its' ability to diagnose auditory neuropathy spectrum disorder combination with Transient Evoked Otoacoustic Emissions test. On the other hand, the test time can be a disadvantage. With technological advancements, a new generation Automated Auditory Brainstem Response has been developed which is faster. The new design of the Automated Auditory Brainstem Response is designed with a coupler that does not contain disposable electrodes is available at an acceptable cost. The aim of this study was comparing the new generation's Automated Auditory Brainstem Response and Transient Evoked Otoacoustic Emissions test by regarding to their test times. The results were then compared with those in previously published literature. Methods: Two hundred and sixty healthy infants were included in the study. The hearing screening of all infants was performed using Transient Evoked Otoacoustic Emissions and Automated Auditory Brainstem Response test devices with new, improved technology. The Ero-ScanTM (Maico, Berlin, Germany) test system was used for the Transient Evoked Otoacoustic Emissions, and the newly designed Maico MB11 BERAphone (Maico-Berlin, Germany) Auditory Brainstem Response screening device with three electrodes in one cap was used for the Automated Auditory Brainstem Response test. Results: Mean age of babies was 60.7+/-51.3 hours, and age range was 4hours-312hours. The test times for the Transient Evoked Otoacoustic Emissions were 13.68+/-9.2s and 14.04+/-9.45, and for the Automated Auditory Brainstem Response, they were 39.15+/-22.2s and 45,25+/-23,9s for the right and left ears respectively. Conclusions: Although the Automated Auditory Brainstem Response test time is statistically longer than the Transient Evoked Otoacoustic Emissions, the amount of time it takes has been significantly shortened by the new technology. This finding enhances the value of the new generation Automated Auditory Brainstem Response technology usage as an initial test for newborn hearing screening.Wo

Chronic lymphocytic leukemia and multiple myeloma in the same patient: Case report

We hereby report the occurrence of a non-secreting multiple myeloma (MM) during the course of chronic lymphocytic leukemia (CLL). A 73-year-old patient developed a non-secreting MM 7 years after the diagnosis of CD5 (+) B cell CLL. He received intermittent chlorambucil and prednisolone therapy. The occurrence of tumors in the frontal bone led to resection of the tissue and histopathologic examination revealed neoplastic plasma cell islands within the CLL infiltration. There was no immunoglobulin or light chain accumulation in the serum. Clonal relationship between these diseases has been shown by comparing the isotypes and idiotype of both the heavy and light chains. In our case, the MM was of non-secreting type and thus we could not establish any relation between the 2 disorders. Such a case has not been reported until now in the literature. CLL and MM in the same patient is a rare occurence and investigation of the transformation event at the molecular level will contribute to our understanding of B-cell ontogenesis