Roseomonas gilardii Bacteremia in a Patient With HbSß0-thalassemia: Clinical Implications and Literature Review.

Roseomonas gilardii is a Gram-negative coccobacillus identified in immunocompromised pediatric patients. A 5-year-old male with a history of HbSß thalassemia status postsurgical splenectomy presented to the emergency department with fever. Blood cultures grew R. gilardii at 63 hours, but the patient had been discharged home at 48 hours. The patient was readmitted for repeat cultures and initiated on meropenem for 10 days as Roseomonas spp. are often resistant to third generation cephalosporins. R. gilardii is a rare cause of bacteremia in immunocompromised patients. Clinicians should consider Roseomonas in slow growing Gram-negative rod bacteremias, and consider meropenem as empiric coverage.

Osteomyelitis and pyomyositis due to Pseudomonas aeruginosa in a child with sickle ß°-thalassemia.

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 describe an 8-year-old boy who presented with high-grade fever and tender, swollen left thigh. There was a history of intramuscular injections in the left thigh. He also had severe anemia, hepatosplenomegaly, and laboratory evidence of hemolysis. Hemoglobin electrophoresis showed sickle ß-thalassemia. Magnetic resonance imaging of the left thigh showed evidence of osteomyelitis with pyomyositis. Surgical drainage of the pus was done and Pseudomonas aeruginosa was isolated. He was treated with intravenous antibiotics for 8 weeks. The child had a protracted course of illness with development of pathologic fracture of the femur. Clinicians need to be aware of Pseudomonas infection as a complication in children with sickle cell disease, as this affects therapeutic decisions, including the choice of antibiotics.

Pneumocystis carinii pneumonia in patients with malignant haematological diseases: 10 years' experience of infection in GIMEMA centres.

A retrospective survey was conducted over a 10-year period (1990-99) among 52 haematology divisions in order to evaluate the clinical and laboratory characteristics and outcome of patients with proven Pneumocystis carinii pneumonia (PCP) complicating haematological diseases. The study included 55 patients (18 with non-Hodgkin's lymphoma, 10 with acute lymphoblastic leukaemia, eight with acute myeloid leukaemia, five with chronic myeloid leukaemia, four with chronic lymphocytic leukaemia, four with multiple myeloma, three with myelodysplastic syndrome, two with myelofibrosis and one with thalassemia) who developed PCP. Among these, 18 (33%) underwent stem cell transplantation; only two received an oral prophylaxis with trimethroprim/sulphamethoxazole. Twelve patients (22%) developed PCP despite protective isolation in a laminar airflow room. The most frequent symptoms were: fever (86%), dyspnoea (78%), non-productive cough (71%), thoracic pain (14%) and chills (5%); a severe hypoxaemia was present in 39 patients (71%). Chest radiography or computerized tomography showed interstitial infiltrates in 34 patients (62%), alveolar infiltrates in 12 patients (22%), and alveolar-interstitial infiltrates in nine patients (16%). Bronchoalveolar lavage was diagnostic in 47/48 patients, induced sputum in 9/18 patients and lung biopsy in 3/8 patients. The diagnosis was made in two patients at autopsy. All patients except one started a specific treatment (52 patients trimethroprim/sulphamethoxazole, one pentamidine and one dapsone). Sixteen patients (29%) died of PCP within 30 d of diagnosis. Multivariate analysis showed that prolonged steroid treatment (P < 0.006) and a radiological picture of diffuse lung involvement (P < 0.003) were negative diagnostic factors.

Severe thalassaemia intermedia: clinical problems in the absence of hypertransfusion.

In many of the parts of the world where thalassaemia is common, the blood supply is inadequate or unsafe, and desferrioxamine is too expensive for routine use. We classify some patients as having 'severe thalassaemia intermedia', i.e. those with moderately severe thalassaemia who can survive without regular transfusions, but who are at risk of many complications which are reviewed here. These include bone deformity and fractures, extramedullary haemopoietic tumours, leg ulcers, autoimmune haemolysis and, especially after splenectomy, thromboembolism and infection. An increase in the quality and safety of the blood supply, and a cheaper and/or oral iron chelator, would enable more of these patients to be treated as thalassaemia major and have improved survival and quality of life.

Diagnóstico diferencial de la microcitemia en pacientes hospitalarios / Differential, diagnosis of the microcytosis in hospitalized patients

En el presente estudio, se pudo comprobar que los índices eritrocitarios VCM (Volumen Corpuscular Medio) y RDW (Amplitud Distribución Eritrocitaria), en unión del computo absoluto de eritrocitos, son indicadores de buena sensibilidad para la clásica B-talasemia (9,4 por ciento) y la anemia por defiencia de hierro (56 por ciento), condiciones que constituyeron más del 65 por ciento de microcitemia encontrada. El nivel de la PP-Zn evidenció en forma rápida la deficiencia de hierro y la correspondiente micrositosis. En los casos considerados como propios de una enfermedad crónica, se demostró una anormalidad de la PP-Zn en aproximadamente la mitad de los casos. Fue evidente que en comparación con anemia ferropriva las B-tal menores resultan ser más microcíticas que hipocrómicas, y que el diagnóstico de la alfa talasemia menor es asunto difícil si se exige para ello la presencia de cuerpos de inclusión de Hb H. Finalmente, se considera necesario ampliar la experiencia que se ha acumulado en torno al uso de los autoanalizadores, en especial para conocer mejor la utilidad del índice RDW en la discriminación de la microcitemia

Antibody to hepatitis C virus in thalassemia patients.

A specific enzyme immunoassay (EIA) for the diagnosis of hepatitis C virus (HCV) infection was developed by recombinant DNA technology. Abbott HCV EIA was used to detect antibody to HCV (anti-HCV) in non-transfused and multiply-transfused thalassemia patients. None of 11 non-transfused patients had anti-HCV but 3 of 52 (5.8%) multiply-transfused patients had anti-HCV. This study showed that the prevalence rate of HCV infection is low in thalassemia patients. However, it is still important to identify hepatitis C virus infected patients in high risk groups because hepatitis C is associated with chronic hepatitis, cirrhosis and hepatocellular carcinoma.

Hallazgos de hemoglobinopatías en un laboratorio de clínica periférica de la Caja Costarricense de Seguro Social / Hemoglobinopathies findings in a peripherical clinic laboratory of the Caja Costarricense de Seguro Social

Se analizaron 23.000 hemogramas de rutina en el Laboratorio de la Clínica Dr. Marcial Fallas de Desamparados, durante el período de noviembre de 1989 a febrero de 1991, encontrándose los siguientes resultados: 97 casos (0,413%) fueron identificados como sospechosos de hemoglobinopatía, basados en el observación microscópica de la morfología de la serie roja tales como microcitosis, hipocromía, basofilia difusa, punteado basófilo, codocitos, drepanocitos y la prueba presuntiva de fragilidad osmótica. Estas muestras fueron enviadas al C.I.H.A.T.A. para realizar una electroforesis de hemoglobina. Se confirmaron 42 resulados positivos (0,178%), los cuales se distribuyeron como sigue: 26 casos de Beta talasemia menor tipo A2 elevada (0.111%), 5 casos de patrón SS (0.021%), 4 casos de patrón As (0.017%), 3 casos de patrón AC (0,013%), 2 casos de patrón SC (0,008%), 1 caso de delta beta talasemia (0,004%), 1 caso de S beta talasemia (0,004%).

Deferoxamine increases the susceptibility of beta-thalassemic, iron-overloaded mice to infection with Listeria monocytogenes.

The effect of the iron chelator deferoxamine (DFO) on resistance to infection with Listeria monocytogenes in mice with a condition analogous to human beta-thalassemia was studied. Intraperitoneal injection of 10 mg DFO resulted in significantly increased mortality when given one, three and six days before infection with L. monocytogenes (for all three time points, p less than 0.02). There were no significant differences in hematocrit, plasma iron, or splenic iron content between the two groups of mice during these time periods. In addition, splenic counts of L. monocytogenes were not significantly higher in DFO-treated compared to saline-treated mice three days after infection. Moreover, background C57Bl/6J mice were not more susceptible to Listeria infection after receiving DFO than were saline-treated controls. In conclusion, acute administration of DFO increases the susceptibility of beta-thalassemic mice to L. monocytogenes. The effect is not seen in background mice and suggests that DFO increases susceptibility to Listeria infection only in animals with iron overload.