Blood disorders

Immune thrombocytopenic purpura (ITP) is an autoimmune disease characterized by the production of antibodies to circulating platelets. Traditionally it has been regarded as a disorder of increased platelet destruction, so therapies have targeted this mechanism, including corticosteroids, IVIG, splenectomy and rituximab. More recently, it has become clear that decreased...

Immune Thrombocytopenic Purpura (ITP) has long been thought to result predominantly from immune mediated acceleration of platelet destruction. As a result, immunosuppressive therapy has traditionally played a central role in the treatment of the disorder. Despite many advances in treatment options chronic ITP that is refractory to available therapy remains...

Taking an excess amount of caffeine (e.g. overdrinking caffeinated beverages) sometimes causes hypokalaemia. Although the detailed mechanism has not been clarified yet, an increased loss of potassium via the urine stream caused by the diuretic action of caffeine is proposed as one of the possibilities. We report the case of...

Background: In neutropenic patients with acute leukemia, acute abdomen which is usually attributed to typhlitis may be encountered. However, the diagnosis of acute appendicitis is rarely described or confirmed. Methods and Materials: A retrospective study of acute appendicitis in adult patients with acute leukemia was conducted at the Riyadh Armed...

The ubiquitin-proteasome-degradation system plays a key role in multiple cellular functions. Its deregulation is associated with the initiation and progression of human diseases including not only solid and hematologic malignancies but also neurologic and autoimmune disorders. This article discusses several novel mechanistic aspects of the ubiquitin-proteasome system. Moreover, it focuses...

Background: Doppler echocardiographic studies of left ventricle (LV) systolic and diastolic function in patients with β-Thalassemia Major (β-TM) had shown different patterns of systolic and diastolic dysfunction. Aim: This cross-sectional study was designed to study the LV systolic and diastolic function in patients with β-TM using Pulsed Doppler (PD)...

Introductory Editorial by Dr Karna Dev Bardhan

Paroxysmal nocturnal hemoglobinuria (PNH) is a potentially life-threatening acquired clonal stem cell disorder resulting in hemolysis and thrombosis. A somatic mutation in the phosphatidylinositol glycan A gene in hematopoietic stem cells results in a deficiency of the complement regulatory proteins, CD55 and CD59. There is a consequent increased sensitivity to...

Anemia resulting from a relative deficiency of erythropoietin commonly complicates chronic kidney disease (CKD). With the introduction of recombinant erythropoietin two decades ago, there has been a dramatic reduction in the need for blood transfusions in CKD patients. Epoetin alpha is a first generation erythropoiesis stimulating agent (ESA) that needs...

Myelodysplastic syndromes (MDS) are marked by progressive cytopenias and risk of transformation to acute myeloid leukemia. Supportive care with transfusions, antibiotics, and hematopoietic growth factors has long been the mainstay of therapy for MDS, given that most patients are not eligible for more intensive chemotherapy. The hypomethylating agent 5-azacitidine (AZA)...

Most patients with hereditary or chronic acquired anemias are dependent on regular red cell transfusions. Untreated iron overload from transfusions is responsible for morbidity and mortality in patients with thalassemia major. However, clinical consequences of parenchymal iron overload have been reported not only in thalassemia major but also in patients...

To assess some simple blood parameters at birth that can be used as a basis to suspect α-thalassemia minor (ATM), a prospective study involving 202 consecutive neonates with MCV of less than 95 fl or less were checked for Hb Barts by HPLC. The group was divided into two, one...

Terje Forslund1, Anna-Mari Koski2, Arvo Koistinen1 and Anu Sikiö3 1Division of Nephrology and 2Division of Endocrinology and 3Division of Haematology, Department of Medicine, Central Finland Health Care District Hospital, Jyväskylä, Finland. Abstract A breakthrough in understanding of mechanisms of bone structure regulation has brought about the introduction...

Khalid A. Al-Anazi1, Asma M. Al-Jasser3, Amal Abdulwahab1, Entezam Sahovic1, Hadeel Almana2 and Mohammed A. Al Fadda2 1Section of Adult Hematology and Hematopoietic Stem Cell Transplant, King Faisal Cancer Centre, King Faisal Specialist Hospital and Research Centre, P.O. Box: 3354, Riyadh 11211, Saudi Arabia. 2Departments of Medicine and Pathology,...

J.A.Olaniyi Department of Haematolgy. University College Hospital, PMB 5116, Ibadan, Nigeria. Abstract This case report illustrates the multiple complications experienced by a sickle cell anaemia patient. Although he enjoyed fairly good health till age 16 years; he subsequently suffered grade four bilateral femoral head necrosis, cerebella infarct...

Juliane Menezes1, Mariana Emerenciano1, Flávia Pimenta2, Gilson Guedes Filho5, Isis Q. Magalhães3, Mariana Sant’Ana1, Marina Lipkin Vasquez4, Ilana Zalcberg Renault4 and Maria S. Pombo-de-Oliveira1 1Programa de Hematologia e Oncologia Pediátricos, Centro de Pesquisa, Instituto Nacional de Câncer, Rio de Janeiro, RJ, Brazil. 2Departamento Medicina Interna, Disciplina Hematologia UFPB, João...

Khalid A. Al-Anazi1, Mahmoud D. Aljurf1, Fahad I. Al-Mohareb1, Laila Al-Dabal2, Mohammed Zaitoni2 and Majed Halim3 1Section of Adult Hematology and Hematopoietic Stem Cell Transplant, King Faisal Cancer Centre, King Faisal Specialist Hospital and Research Centre, P.O. Box: 3354, Riyadh 11211, Saudi Arabia. Sections of 2Pulmonary Medicine and 3Infectious...

Thomas F. Kresina1, Diana Sylvestre2, Leonard Seeff3, Alain H. Litwin4, Kenneth Hoffman1, Robert Lubran1 and H. Westley Clark1 1Center for Substance Abuse Treatment, Substance Abuse and Mental Health Services Administration, Rockville, MD. 2Department of Medicine, University of California, San Francisco and Organization to Achieve Solutions In Substance...

Pauline Brice, Marc André², Patricia Franchi-Rezgui, Irène Biasoli, C.Hennequin Hôpital Saint Louis AP-HP, services d’hématologie et radiothérapie, Paris, France. ²Hôpital Notre Dame, Charleroi, Belgique. Abstract In patients with Hodgkin lymphoma (HL) a continued improvement in outcome with a high cure rate is observed but with an increased...

Shigeru Tsuboi Infectious and Inflammatory Disease Center, Burnham Institute for Medical Research, La Jolla, California 92037, U.S.A. Abstract The Wiskott-Aldrich Syndrome (WAS) is an X chromosome-linked immunodeficiency disorder. The most common symptom in WAS is bleeding. Several clinical investigations indicate that low platelet counts and defective...