Sideroblastic anaemia as a diagnostic filch point of melas [myoencephalopathy, lactic acidosis and stroke like episodes]

Sideroblastic anaemia includes a heterogeneous group of conditions characterized by decreased heme synthesis and mitochondrial iron overload. It is diagnosed by the presence of ringed sideroblasts in the bone marrow aspirate and association of this form of anemia with various mitochondrial dysfunction disorders can be a catch point to reach an ultimate diagnosis in these disorders as reported in this case of myoencephalopathy, lactic acidosis, and stroke-like episodes [MELAS] syndrome

Sideroblastic Anaemia-A Hitherto Unrceognized cause of unexplained Anaemia

Objective: To assess the clinicopathological heterogeneity of sideroblastic anemia disorders characterized by the presence of ring sideroblasts in the bone marrow

Study Design: Descriptive study

Place and Duration of Study: Study was conducted at department of Pathology, Combined Military Hospital Kharian, from Apr 2014 to Oct 2016

Material and Methods: A total of ten patients diagnosed as having sideroblastic anemia [SA] on cytomorphological basis were included in the study. After clinical examination of the patients, blood samples were analyzed on Sysmex KX21 Haematology analyzer. Blood and bone marrow aspiration slides were stained with leishman's stain to study red cell morphology and aspects of haematopoiesis. Assessment of iron stores was done using Perl's staining technique. Data was collected and analyzed using SPSS 16.0 version

Results: Age range of the SA patients varied from 14-65 years and male to female ratio was 1.3:1. Clinical features included weakness, malaise, easy fatigability, fever, bleeding complications, pallor, splenomegaly and syndrome specific features in 2 patients. MCV ranged from 66 fl to 94 fl. Dimorphic red cell morphology which is considered an important feature of SA was not observed in any patient. Other cytopenias were also noticed. Dysplasia was observed in 4 patients. One patient was confirmed as having secondary SA due to lead poisoning

Conclusion: Clinicopathological features of SA are variable and it is inappropriate to associate SA with any specific age group, gender, red cell indices or morphology. Prompt recognition of SA with its accurate categorization and specific treatment can avoid undue suffering of the patients as well as their relatives

Histopathological outcome of pancytopenia cases on bone marrow trephine biopsy

Objective: To determine the histological outcome of pancytopenia cases on bone marrow trephine biopsy and to see the frequency of various causes of pancytopenia in our population

Study Design: Descriptive study

Place and Duration of Study: Pathology department, Combined Military Hospital [CMH], Kharian [Pakistan]. One year [Jan 2015-Dec 2015]

Material and Methods: Two hundred bone marrow trephine biopsies were done in one year [2015], out of which 40 were done for evaluation of pancytopenia. The criteria for diagnosis of pancytopenia were; haemoglobin less than 10 g/dl, total leukocyte count [TLC] less than 4.0 x 109/1 and platelet count less than 100,000 x 109/1. Patients with pancytopenia secondary to drugs, chemotherapy and radiotherapy were excluded from the study

Trephine biopsies showing marked crushing and having inadequate material were also excluded from the study. Biopsies were processed, slides made and examined under light microscope by haematologist and histopathologist. Frequencies of various causes of pancytopenia diagnosed on histopathology were calculated. The findings were analyzed by using SPSS version 10.0

Result: Out of 40 cases of pancytopenia, male to female ratio was 3:2. The age range was between 1 year to 75 years. Histopathological analysis of bone marrow trephine biopsies revealed megaloblastic anaemia as the most common cause of pancytopenia [30%], followed by aplastic anaemia [25%] and hypersplenism [15%]

Conclusion: Megaloblastic anaemia is the most common cause of pancytopenia in our population as compared to aplastic anaemia mentioned in most of the international studies. This indicates prevalence of nutritional deficiency in our population and megaloblastic anaemia must be kept at top of list while evaluating pancytopenia cases. Early diagnosis and treatment of megaloblastic anaemia will prevent any further complication of this disease

Efficacy of parenteral iron as compared to oral iron in the treatment of iron deficiency anemia in children

To compare the efficacy of parenteral iron as compared to oral iron in the treatment of iron deficiency anemia in children presenting at CMH Murree. Randomized control trail [RCT] Departments of Paediatrics and Pathology CMH Murree from August 2009 to March 2010. Forty one children diagnosed with iron deficiency anemia on the basis of hemoglobin, red cell indices and morphology and serum iron levels were included. Patients were randomized into two groups with group I of 18 receiving oral ferrous sulphate and group II of 23 receiving parenteral iron sorbitol therapy. Response was assessed by reticulocyte count at 1 week, change in hemoglobin, MCV and serum iron levels at 4 weeks post treatment. The reticulocyte response at 1 week time was significantly more with intramuscular iron as compared to oral iron. Mean hemoglobin change and serum iron level increase were also significantly more with intramuscular iron therapy. Mean MCV level change was insignificant between the two modalities. Parenteral intramuscular iron sorbitol treatment with better compliance shows early improved responses in children with iron deficiency anemia as compared to oral ferrous sulphate therapy and should be used as a preferred modality for treatment

Frequency of bleeding disorders diagnosed at armed forces institute of pathology Rawalpindi

To determine the frequency of bleeding disorders diagnosed at Armed Forces Institute of Pathology, Rawalpindi [AFIP Rwp]. Descriptive study. Department of Hematology, AFIP Rwp from January 2006 to June 2009. A total of 1836 patients of bleeding diathesis were included in the study. Hess test was done to investigate the vascular defects. Bleeding Time [BT] was done to screen platelet function defects. The 'clotting screen' and mixing studies were done to detect coagulation protein defects. Clot solubility test was performed to screen factor XIII deficiency. Out of 1836 patietns of bleeding diathesis 435 [23.7%] were diagnosed as having haemostatic defects. Out of these 435 patients 273 [62.8%] had coagulation factor deficiency, 81 [18.6%] had platelet function defects and 81 [18.6%] had vWF deficiency. Among the 273 coagulation factor deficiency patients, factor VIII deficiency was in 121 [44.3%], factor IX deficiency in 32 [11.7%], factor V deficiency in 18 [6.6%], factor XIII deficiency in 15 [5.5%], factor VII deficiency in 12 [4.4%], factor X deficiency in 9 [3.3%], factor I deficiency in 8 [2.9%] and factor II deficiency was in 3 [1.1%]. Multiple factor deficiency was 55 [20.1%]. No defects of vasculature were identified. Coagulation factor deficiencies, with factor VII deficiency being the commonest are the most frequent bleeding disorders. Platelet function defects and vWF deficiency also comprise significant proportion of the bleeding disorders