Frequency of Paroxysmal Nocturnal Hemoglobinuria Clone in Turkish Myelodysplastic Syndrome Group

Aim:Retrospective, cross-sectional, observational study to examine the frequency and features of paroxysmal nocturnal hemoglobinuria (PNH) clones in patients with myelodysplastic syndrome (MDS).Methods:Data were analyzed from the medical files of 41 MDS patients diagnosed and followed up in the hematology department at a referral center between 2006 and 2017. Descriptive data, cytogenetic and hematologic characteristics, prognostic features and PNH clone sizes were assessed. PNH clone sizes were evaluated using the fluorescently labeled inactive toxin aerolysin method.Results:The study population comprised 22 (53.7%) female and 19 (46.3%) male patients with confirmed MDS; the mean age of the patients was 68.20±9.84 years (range, 45-85). PNH clones were detected in eight (19.5%) patients. The number of patients with a PNH clone size of >10%, >1%, >0.1% and >0.01% was one, one, one and eigth, respectively (p<0.001 for all subgroups).Conclusion:These data indicate that PNH clones exist in approximately one-fifth of MDS patients. Further studies on a more extensive cohort are required to better understand the pathophysiological and clinical relationships between MDS and PNH

The Prevalence of Paroxysmal Nocturnal Hemoglobinuria Clone in Adult Patients with Idiopathic Pulmonary Hypertension

Aim: Paroxysmal nocturnal hemoglobinuria (PNH) a is a clonal disorder that may lead to several conditions such as thromboses, Budd-Chiari syndrome, renal failure, impotence, and pulmonary hypertension (pHT). Since the presentation of PNH may be occult, monitoring for clonal evolution is recommended in certain situations including aplastic anemia, Myelodysplastic syndrome, and unexplained cytopenia, and thrombosis. The prevalence of PNH clone in patients with idiopathic pHT is unknown. We designed a study to determine the prevalence of PNH clone in patients with idiopathic pHT, since it may be the first isolated presentation of the disease. Methods: A total of 45 patients with pHT were screened for PNH clone by proaerolysin conjugated with fluorescein. Results: Only two out of 45 patients had elevated lactate dehydrogenase (LDH) levels at presentation. PNH clone was detected in none of the patients. Conclusion: Screening for PNH clone in patients with pHT, who have normal LDH levels is unnecessary

Evaluation of Hypogammaglobulinemia in Chronic Lymphocytic Leukemia Patients and Its Relation to Poor Prognostic Factors

Aim:Chronic lymphocytic leukemia (CLL) is a heterogenous disease with variable clinical course. Rai staging system is used for at least 40 years to predict prognosis and need for treatment but more prognostic factors are needed. Infections have been known to have a significant impact on patients with CLL. It is postulated that hypogammaglobulinemia accounts for the high infection rate. The severity of hypogammaglobulinemia is correlated with disease stage and duration.Methods:Data of patients who were diagnosed with CLL according to the the National Cancer Institute Working Group criteria were analyzed retrospectively. The patients were classified according to absolute lymphocyte count, immunoglobulin (Ig) levels, Rai stage, organomegaly, mass lymphadenopathy, lymphocyte doubling time (LDT), presence of B symptoms and treatment status.Results:We found that low IgA levels were associated with LDT and splenomegaly indicating disease burden and activity. The patients with low IgA and IgM levels received more treatment than others with normal Ig levels.Conclusion:In our study, we could not find a significant relationship between the stage and Ig levels but low IgA and IgM levels were significantly associated with need for treatment. Therefore, we suggest measuring IgA and IgM levels which is a simple and inexpensive test, to predict which patients may need treatment and should be observed closely

Evaluation of Hypogammaglobulinemia in Chronic Lymphocytic Leukemia Patients and Its Relation to Poor Prognostic Factors

Aim: Chronic lymphocytic leukemia (CLL) is a heterogenous disease with variable clinical course. Rai staging system is used for at least 40 years to predict prognosis and need for treatment but more prognostic factors are needed. Infections have been known to have a significant impact on patients with CLL. It is postulated that hypogammaglobulinemia accounts for the high infection rate. The severity of hypogammaglobulinemia is correlated with disease stage and duration

Evaluation of Insulin-like Growth Factor-1 and Insulin-like Growth Factor Binding Protein-3 Expression Levels in Patients with Chronic Lymphocytic Leukemia

Objective: Chronic lymphocytic leukemia (CLL) is a disease of nonproliferating and mature-appearing B lymphocytes. Insulin-like growth factor-1 (IGF-1) is a small peptide hormone and has mitogenic and antiapoptotic effects, and insulin-like growth factor binding protein-3 (IGFBP-3) has antiproliferative effects on cells. In this study, we investigated plasma levels of both IGF-1 and IGFBP-3 in patients with CLL compared with controls, and we compared these plasma levels according to prognostic factors

Purtscher-Like Retinopathy Associated with Atypical Hemolytic Uremic Syndrome

A 25-year-old woman presented with acute bilateral blurred vision and history of headache, dizziness, and syncope for three days. Her visual acuity was 20/60 in both eyes. Fundoscopy revealed multiple bilateral peripapillary yellow-white patches like cotton wool spots, intraretinal hemorrhages and macular edema. The patient was diagnosed with Purtscher-like retinopathy based on the retinal findings and lack of trauma history. She was urgently admitted to the nephrology clinic due to thrombotic microangiopathy findings (hemoglobinemia, thrombocytopenia, and acute renal failure). After excluding thrombotic microangiopathy, the patient was diagnosed with atypical hemolytic uremic syndrome (aHUS) with the clinical and laboratory findings. Eculizumab treatment was added to hemodialysis and plasmapheresis therapy. Three months after starting treatment, retinal lesions regressed and visual acuity increased to 20/20 in both eyes. To the best of our knowledge, this is the first reported case of Purtscher-like retinopathy associated with aHUS