Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired, rare clonal blood disorder, characterized by chronic intravascular hemolysis, bone marrow failure, renal failure and pulmonary hypertension, and a heightened risk of thrombotic complications. PNH etiology is an Υ-linked gene somatic mutation of the phosphatidylinositol glycan class ΐ (PIG-A ), that results in deficiency of the glycosylphosphatidylinositol anchor structure responsible for fixing a wide spectrum of proteins on blood cell membranes, absence of these proteins, particularly CD55 and CD59, dysregulates the complement on cell membranes and results in significant chronic complement-mediated hemolysis. Early diagnosis of PNH is crucial for effective disease management. However, the heterogeneity of clinical symptoms and rarity of this disease usually results in untimely diagnosis, severe disability of patients, and increased risk of fatal complication. These recommendations are formulated by a panel of experts from the gulf cooperation countries. This information reflects their experience and to assist specialists looking after PNH patients, including hematologists, nephrologists, dialysis specialists, gastroenterologists, cardiologists, and surgeons.

Context: Pancytopenia is not a disease but a clinico-hematological entity encountered in clinical practice. A prompt intervention is required to avoid complications, which can occur in these patients. The relevant clinical history and complete hematological workup is required to treat patient of pancytopenia. The severity of pancytopenia and the underlying pathology determines the management and prognosis. Aims: To diagnose different conditions producing pancytopenia on the basis of clinical, hematological and bone marrow studies. To estimate the frequency of different diseases producing pancytopenia. Subjects and Methods: The study was conducted in Department of Hematology of our college in capital city of India. A prospective study was carried out among 100 consecutive patients with pancytopenia. Blood samples of the patients were analyzed for red cells, white cells and platelets morphology along with presence and absence of immature cells and abnormal cells. In bone marrow examination, morphology of all cells lineage, cellularity, parasite and abnormal cells were scrutinized. Trephine biopsy was done where ever indicated. Results: The age of patients ranged from 5 to 80 years. 74% of patients in this study were vegetarian. The most common cause of pancytopenia was megaloblastic anemia (66%) followed by aplastic anemia (18%), malaria (6%), kala-azar (4%), acute myeloid leukemia (2%), multiple myeloma (2%), myelodysplastic syndrome (1%), and tuberculosis (1%). Conclusions: The present study concludes that detailed primary hematological investigations along with bone marrow examination in cytopenic patients is helpful for understanding disease process, to diagnose or to rule out the causes of pancytopenia. It is also useful in planning further investigations and management.

Context: Role of youngsters in voluntary blood donation (VBD) is crucial to meet the demand of safe blood. Therefore understanding the various factors contributing to knowledge, attitude, and practice of VBD among college students is important. Aims: The present study aims to assess the level of knowledge, attitude, and practice regarding blood donation among the health care students. Settings and Design: Cross-sectional study done at Anand from July 2012 to December 2013. Subjects and Methods: A cross-sectional study was conducted among 1000 health care medical students at Anand, Gujarat, India using a structured survey questionnaire. Statistical Analysis Used: The data were tabulated, and master chart was prepared. Analysis was performed by using the percentage. Results: Overall knowledge on blood donation among respondents was 37.3%, majority of the participants (78.7%) never donated blood in which 43.7% of the nondonors had negative attitude like blood donation leads to weakness (10.2%), anemia (25.5%), and 8.0% of nondonors felt that blood donation leads to reduced immunity. Majority of nondonors showed a positive attitude (53.1%) by expressing their willingness to donate blood if they were asked to donate blood (35.3%), about 9.8% of the nondonors don't know the importance of blood donation and 11.2% of nondonors don't know where to donate blood. In this study, 11.5% of students had donated blood only once, in which 38.8% of blood donors said no privacy was provided while donation, few (12.2%) had experienced some discomfort after donation and 50.4% of respondent said that they have not tried again. Conclusions: The present study recommends that even health care students' needs to be educated about the importance, awareness and motivation of blood donation on a regular basis and addressing the problems faced, while blood donation will strengthen the recruitment and retention of blood donors to donate blood on a regular basis.

Objective: Many studies have reported an association between chronic immune thrombocytopenia and Helicobactor pylori infection. Significant improvement in platelet count is seen after eradication therapy. We looked for the frequency of H. pylori infection in immune thrombocytopenia and clinico-hematological features. Material and Method: A prospective case-control study was conducted from January 2009 to December 2011. One hundred and ten chronic immune thrombocytopenic patients and 40 control subjects were enrolled. H. pylori infection was documented by H. pylori stool antigen enzyme immunoassay method. Results: H. pylori infection in immune thrombocytopenic patients was seen in 45 cases (40.9%) and in 24 (60%) controls; not significant statistically. No significant differences were seen in H. pylori positive and negative patients with respect to maternal characteristics and clinical features except for mean platelet count, which was lower in H. pylori negative group (P = 0.035). Conclusion: Unlike many related studies, we found no significant difference between the frequencies of cases versus controls. Degree of thrombocytopenia appears more marked in H pylori negative patients.

Introduction: Thrombocytopenia is a common hematological abnormality observed in hospitalized patients. Objective: The present study was conducted to determine the prevalence of thrombocytopenia and its underlying causes. Patients and Methods: A retrospective analysis was performed on 221 thrombocytopenia patients (platelet count <100 × 10 ⁹ /L) admitted to a single center (Al Qassimi Hospital-United Arab Emirates, Sharjah) between the years 2005 and 2010, and the demographics, associated conditions, therapy and outcome assessed. Results: Median age at diagnosis was 39 years (range 0-90 years), including 18.5% children. The majority of patients presented with platelet counts ≥71 × 10 ⁹ /L. Thrombocytopenia was more common in younger women (20-40 years); the proportion of men was greater in the elderly population. Thrombocytopenia was associated in 28.5% of the patients with infections, in 14% with pregnancy, in 10.4% with cardiac disease, in 10.0% with surgical interventions and in 7.7% with liver/kidney disease. Immune thrombocytopenia was diagnosed in 3.6% of the patients. Spontaneous recovery was observed for 72.5% of the untreated patients, and 71.9% of the patients receiving antibiotics to treat underlying infections subsequently recovered. Conclusion: The reason for low platelet counts may be multi-factorial for many patients and not restricted to a single underlying condition. Standard treatment options are generally effective, but newer treatment modalities may be explored for patients which fail to respond.

Small bowel infarction is a rare and fatal complication of invasive aspergillosis following chemotherapy in acute myeloid leukemia (AML) patients. We describe a case of an adult patient with an AML who developed appendicitis and intestinal infarction secondary to aspergillosis following anti-leukemic chemotherapy. The diagnosis was made histologically at laparotomy. The patient died later despite antifungal therapy.

We are reporting a case of 70-yr-old gentleman who presented to dermatology clinic with painful red rash which was confirmed to be a sweet's syndrome by biopsy . Further work up showed IgA myeloma. The patient showed good response to systemic and topical steroid. This case illustrate the need of considering paraneoplastic phenomenon in elderly with sweet's syndrome . Sweet's syndrome has been rarely reported as paraneoplastic phenomenon in of multiple myeloma.