BACKGROUND Patients with the Shwachman-Diamond syndrome often develop hematologic complications. No risk factors for these complications have so far been identified. The aim of this study was to classify the hematologic complications occurring in patients with Shwachman-Diamond syndrome and to investigate the risk factors for these complications. DESIGN AND METHODS One hundred and two patient...

BACKGROUND The aim of this study is to investigate the hematological manifestations of human immunodeficiency virus (HIV) infection, the risk factors for cytopenia, and the effect of highly active anti-retroviral therapy (HAART) on cytopenia. METHODS Medical records of patients treated for HIV at the Seoul National University Hospital from January 2005 to March 2010 were retrospectively revie...

OBJECTIVE Systemic lupus erythematosus (SLE) may affect a number of systems, with the hematological system being one of the most common. Our aim is to determine the existence of cytopenia at diagnosis or during follow-up of our SLE patients as well as the associated factors. MATERIAL AND METHODS A cohort of SLE patients that had been followed-up in the Department of Rheumotology from 1998 to ...

Autoimmune lymphoproliferative Syndrome (ALPS) is a rare inherited disorder of apoptosis. It usually presents with chronic lymphadenopathy, splenomegaly, and symptomatic cytopenia in a child. Herein, we report a 14-year-old boy with symptoms misdiagnosed as hemophagocytic lymphohistiocytosis who was treated before ALPS was diagnosed for the patient. This case should alert pediatricians to consi...

autoimmune lymphoproliferative syndrome (alps) is an uncommon nonmalignant lymphoproliferative disease which is characterized by chronic, persistent or recurrent lymphadenopathy, splenomegaly, hepatomegaly, immune cytopenia, hypergammaglobinemia and increased risk of lymphoma. we report a 2-year old boy with hepatosplenomegaly as first presentation. petechial and purpuric rashes with massive ce...

Refractory cytopenia of childhood is the most common type of childhood myelodysplastic syndrome. Because the majority of children with refractory cytopenia have a normal karyotype and a hypocellular bone marrow, differentiating refractory cytopenia from the immune-mediated bone marrow failure syndrome (very) severe aplastic anemia can be challenging. Flow cytometric immunophenotyping of bone ma...

Background and Aims: Transient cytopenia due to infectious agents is frequent in childhood. Clinical picture changes between mild to life-threatening conditions. In this study, viral aetiology of previously healthy children admitted with viral symptoms and cytopenia were evaluated. Methods: Children admitted from January to April 2015 were included. Children with chronic diseases and previous n...

Myelodysplastic syndromes (MDS) are a heterogeneous group of neoplastic diseases the hematopoietic cells manifested by ineffective hematopoiesis and tendency to transform into acute myeloid leukemia. MDS should be considered in differential diagnosis cytopenia, especially elderly. This article presents recommendations experts Polish Adult Leukemia Group (PALG) for myelodysplastic syndromes. We ...