beta-thalassemia/fever

Globin chain imbalance and tissue hypoxia are important determinants of the clinical severity of thalassemias. Phenotypic expression may be further modified by interactions between alpha- and beta-thalassemia defects. We retrospectively and prospectively studied the clinical and hematologic features

An 8-month-old male infant with severe anemia and thrombocytopenia was brought to our hospital due to fever and pallor. The physical examination on admission showed pale conjunctivae, skin rash, lymphadenopathy and hepatosplenomegaly. Subsequent laboratory studies showed pancytopenia, impaired liver

BACKGROUND Post-transplant lymphoproliferative disorder (PTLD) is the most common form of lymphoproliferation in childhood and is associated with significant morbidity and mortality. In this report we reviewed the case of a pediatric patient who experienced PTLD after allogeneic hematopoietic stem

Beta-thalassemia, which results from a reduced production of beta-globin chain of hemoglobin, is a common single gene disorder with an extremely heterogeneous clinical picture. Its presentation may vary from mild anemia in beta-thalassemia minor to severe and life-threatening anemia in

It appears that delayed hemolytic transfusion reactions may occur several days after the administration of donor red cells is true even though they have been shown to be compatible in cross match tests by the antiglobulin technique. A specific case was observed in our center, which confirms the

Sickle cell osteomyelitis is usually due to Salmonella or Staphylococcal etiology. Pseudomonas as a cause of sickle cell osteomyelitis is rare. Similarly, pyomyositis is a rare complication in children with sickle cell disease and few cases have been reported, predominantly due to Staphylococcus. We

Vibrio vulnificus is a halophilic Vibrio that has been isolated repeatedly from sea-water and shellfish during the warm months of the year. It's a virulent pathogen for men and is frequently associated with overwhelming infections including sepsis, gangrene of extremities and high mortality rate. We

A 29-year-old male with transfusion-dependent β-thalassemia major (β-TM), splenectomized and on chelation therapy with deferiprone (DFP or L1) due to heart and liver hemosiderosis, presented with high fever and agranulocytosis. Deferiprone was discontinued and a broad spectrum antibiotic therapy was

Cytomegalovirus (CMV) rarely causes disease in immunocompetent individuals but may cause severe disease in immunocompromised patients. We report the case of a young woman who had undergone multiple transfusions and splenectomy for homozygous β-thalassemia. She presented with prolonged fever and

OBJECTIVE To report a case of hyperchloremic metabolic acidosis in a patient with beta thalassemia major secondary to treatment with deferasirox due to iron overload. METHODS A 58-year-old white female with beta thalassemia major was admitted with fever, fatigue, abnormal liver function test

Acute chest syndrome (ACS), characterized by fever, respiratory symptoms, and new pulmonary infiltration, is a serious complication of sickle cell disease (SCD). Regardless of the etiology, the conventional treatment options for ACS include empirical antibiotic therapy, the administration of

There are limited data concerning safety and immunogenicity of Haemophilus influenzae type b polysaccharide vaccine in children with sickle cell disease. Ninety-eight patients with sickle cell disease (65 with SS phenotype, 23 with SC phenotype, and 10 with S beta-thalassemia) 1.5 to 20 years of age

Langerhans' cell histiocytosis (LCH) is an uncommon disorder of childhood, formerly referred to histiocytosis X. A significant proportion of children with disseminated disease may undergo progression to a fatal outcome despite chemotherapy with single or multiple agents. Only six cases of LCH

A 12-year-old girl with beta-thalassemia hemoglobin E disease received a marrow transplant from her HLA-identical elder brother in July 1995. She had previously been treated by repeated blood transfusions. Conditioning included busulfan 16 mg/kg for 2 days and cyclophosphamide 120 mg/kg for 2 days.

Renal abscesses are rare conditions in children, but they must be remembered in differential diagnosis of fever and abdominal pain. The authors report two paediatric cases with unusual presentation. Case 1: a 15-year-old girl was admitted following a period of fever, vomiting and left hypochondrium