Chediak-Higashi syndrome (CHS) is a very rare autosomal recessive immunodeficiency disorder characterized by partial albinism, recurrent pyogenic infections, and large granules in all granule-containing cells. The author describes a Thai girl who was the first case of CHS in Thailand. She presented in the accelerated phase of CHS, which leads to repeated infections and bleeding, often resulting...

human brucellosis is common in developing countries and is a multi-system disease with a broad spectrum of clinical manifestations. cutaneous lesions associated with brucellosis have been rarely reported in the literature. here we present a case of a 32-year-old man with history of consumption of cheese made from raw milk seen with occurrence of pancytopenia and diffuse maculopapular rash durin...

Pancytopenia is characterized by a decreased number of at least two blood cell lines. Pancytopeniamay progress acutely, such as with decreased blood cell counts in fulminant sepsis, disseminated intravascular coagulation, or rapid hemolysis. Alternatively, pancytopenia may evolve insidiously over weeks to months. In this review we focus on the latter group of acquired and congenital pancytopeni...

Hematopoietic stem cells (HSCs) inhabit distinct microenvironments within the adult bone marrow (BM), which govern the delicate balance between HSC quiescence, self-renewal, and differentiation. Previous reports have proposed that HSCs localize to the vascular niche, comprised of endothelium and tightly associated perivascular cells. Herein, we examine the capacity of BM endothelial cells (BMEC...

FDE: fixed drug eruption INTRODUCTION Mycophenolate is a reversible inhibitor of inosine monophosphate dehydrogenase, which plays an important role in the production of DNA. This drug is commonly used in several autoimmune and inflammatory conditions, including connective tissue diseases, immunobullous disease, and atopic dermatitis, and in the setting of organ transplantation. Mycophenolate is...

For many years hypoplastic or aplastic anemia has been identified with a confusing group of pancytopenias which are refractory to treatment. Recently The Committee for Clarification of the Nomenclature of Cells and Diseases of the Blood and Blood-forming Organs has redefined the term hypoplastic anemia and discarded the term aplastic anemia."3 Hypoplastic anemia is defined by this committee as ...

Previously, we described a group of patients with hemocytopenia who did not conform to diagnostic criteria of known hematological and nonhematological diseases. Most patients responded well to adrenocortical hormone and/or high-dose intravenous immunoglobulin treatment, indicating that cytopenia might be mediated by autoantibodies. Autoantibodies were detected on the membrane of various bone ma...

    Human brucellosis is common in developing countries and is a multi-system disease with a broad spectrum of clinical manifestations. Cutaneous lesions associated with brucellosis have been rarely reported in the literature. Here we present a case of a 32-year-old man with history of consumption of cheese made from raw milk seen with occurrence of pancytopenia and diffuse maculopapular rash...

Primary myelofibrosis (PMF) is a rare chronic BCR-ABL1-negative myeloproliferative neoplasm characterized by progressive bone marrow fibrosis, inefficient hematopoiesis, and shortened survival. The clinical manifestations of PMF include splenomegaly, consequent to extramedullary hematopoiesis, pancytopenias, and an array of potentially debilitating constitutional symptoms. The diagnosis is base...

An 80-year-old male presented with worsening fever, chills and night sweats 10 days after he received his fifth intravesical BCG instillation for his recurrent low-grade transitional cell carcinoma of the bladder. Review of systems was remarkable for mild dysuria and hematuria. Physical examination revealed a temperature of 102F. He had no hepatosplenomegaly or lymphadenopathy. Laboratory evalu...