Atrofia branca de Milian / Atrofie blanche

A vasculopatia livedoide é uma doença cutâneo-vascular que surge devido à oclusão trombogênica de vasos da derme. Apresenta-se por meio de máculas ou pápulas eritematosas e purpúricas, em membros inferiores, que podem levar à ulceração dolorosa crônica e recorrente. Com a evolução, pode haver cicatrização, o que leva ao aparecimento de áreas de fibrose e cicatrizes atróficas, irregulares e esbranquiçadas, dando nome à patologia. Relata-se o caso de uma paciente com vasculopatia livedoide de acometimento de membros inferiores. (AU)

Livedoid vasculopathy is a vascular skin disease resulting from thrombogenic occlusion of dermal blood vessels. It presents with erythematous, purpuric macules or papules on the lower limbs, which can lead to chronic and recurrent painful ulceration. With its progression, there may be scarring leading to the appearance of areas of fibrosis and atrophic, irregular, and whitish scars, which gave this name to the pathology. The case of a patient with livedoid vasculopathy affecting the lower limbs is reported. (AU)

Nature and severity of dental malocclusion in children suffering from transfusion-dependent (-thalassemia major

ABSTRACT Objective: To evaluate the prevalence and severity of malocclusion in children suffering from β-thalassemia and to assess orthodontic treatment need using Grainger's Treatment Priority Index (TPI) and index of orthodontic treatment need (IOTN)-dental health component (DHC). Methods: A cross-sectional study was conducted on 200 transfusion-dependent children diagnosed with homozygous β-thalassemia and 200 healthy school children aged 11-17 years. The TPI and IOTN-DHC data was recorded for both groups. Total TPI score for each subject was calculated and graded according to malocclusion severity estimate (MSE). Independent sample t-test was used to compare mean TPI scores, overjet and overbite between thalassemic and healthy children. Chi-square test was used to compare the frequency of IOTN-DHC grades, Angle's classification, and MSE grades between thalassemic and healthy children. Results: The most prevalent malocclusion was Class I in normal children (67.5%) and Class II in thalassemic children (59%). The mean overjet and overbite were significantly (p<0.001) greater in thalassemic children than in healthy children. Severe tooth displacements were 3.5 times greater in thalassemic children, compared to controls. A greater proportion of thalassemic children were in IOTN grades 3 and 4, compared to the controls (p<0.001). MSE grades 4 and 5 were significantly (p<0.001) more prevalent in thalassemic children, compared to the controls. Conclusion: There is a high prevalence of Angle's Class II malocclusion in thalassemic children. Majority of these children are categorized in higher grades of IOTN-DHC and TPI-MSE, showing a great severity of malocclusion and high orthodontic treatment needs.

RESUMO Objetivo: Avaliar a prevalência e gravidade da má oclusão em crianças que sofrem de beta-talassemia e mensurar a necessidade de tratamento ortodôntico usando o Índice de Prioridade de Tratamento (IPT) de Grainger e o Índice de Necessidade de Tratamento Ortodôntico (Index of Orthodontic Treatment Need - IOTN) - Componente de Saúde Dental (Dental Health Component - DHC). Métodos: Um estudo transversal foi conduzido com 200 crianças com diagnóstico de beta-talassemia homozigótica dependentes de transfusão e 200 crianças saudáveis em idade escolar, entre 11 e 17 anos. Os dados do IPT e do IOTN-DHC foram documentados para ambos os grupos. A pontuação total do IPT para cada sujeito foi calculada e classificada de acordo com a Estimativa de Severidade da Má oclusão (ESM). O teste t para amostras independentes foi usado para comparar os escores médios de IPT, sobressaliência e sobremordida, entre as crianças saudáveis e as com talassemia. O teste qui-quadrado foi usado para comparar a frequência dos escores do IOTN-DHC, a classificação de Angle e os escores do ESM entre crianças com beta-talassemia e crianças saudáveis. Resultados: A má oclusão mais prevalente foi a Classe I em crianças normais (67,5%) e a Classe II em crianças com beta-talassemia (59%). Os valores médios de sobressaliência e a sobremordida foram significativamente (p< 0,001) maiores em crianças com beta-talassemia do que em crianças saudáveis. Os deslocamentos dentários graves foram 3,5 vezes maiores em crianças com beta-talassemia em comparação com os controles. Uma proporção maior de crianças com beta-talassemia estava nos escores 3 e 4 do IOTN em comparação com os controles (p <0,001). Os escores 4 e 5 de ESM foram significativamente (p< 0,001) mais prevalentes em crianças com beta-talassemia em comparação com os controles. Conclusão: Há uma alta prevalência de má oclusão de Classe II de Angle em crianças com beta-talassemia. A maioria dessas crianças é categorizada em escores superiores de IOTN-DHC e IPT-ESM, mostrando uma grande gravidade de má oclusão e alta necessidade de tratamento ortodôntico.

Beta talasemia y embarazo / Beta thalassemia and pregnancy

La talasemia es un desorden congénito hemolítico causado por una deficiencia parcial o completa de la síntesis de las cadenas alfa o beta de las globinas de la hemoglobina. Se manifiesta en una amplia gama de cuadros clínicos que van desde la muerte intrauterina hasta la microcitosis asintomática sin anemia. El depósito de hierro constituye la complicación más importante de la talasemia y su mayor preocupación en el manejo. El embarazo en mujeres con talasemias deviene un grave problema de salud que exige una atención diferenciada y multidisciplinaria. Para esta afección no existe tratamiento específico definitivo. El arsenal médico existente solo puede manejar los efectos y no la causa. El objetivo del trabajo es mostrar algunos aspectos relacionados con las complicaciones que pueden aparecer en esta entidad cuando se asocia al embarazo, la discusión del problema clínico, el diagnóstico y la importancia de la prevención y del consejo genético oportuno en este grupo de pacientes. Presentamos una gestante de 26 años de edad con edad gestacional de 13,5 semanas de piel negra, con antecedentes patológicos personales de S-b Talasemia. Desde muy pequeña presenta dolor en extremidades a predominio en miembros superior derecho e inferior izquierdo, fijo, de intensidad moderada a severa, que no responde a los analgésicos, acompañado de dolor en hipogastrio que se intensifica en los días siguientes. Requiere realizar dos exanguíneo transfusiones. Se reafirma la importancia de la visita preconcepcional para educar preventivamente a la población antes del embarazo para evitar complicaciones(AU)

Thalassemia is a congenital hemolytic disorder caused by a partial or complete deficiency of the synthesis of the alpha or beta chains of hemoglobin globins. It manifests itself in a wide range of clinical conditions ranging from intrauterine death to asymptomatic microcytosis without anemia. Iron deposition is the most important complication of thalassemia and its major management concern. Pregnancy in women with thalassemias becomes a serious health problem requiring differentiated and multidisciplinary attention. There is no definitive specific treatment for this condition. The existing medical arsenal can only handle the effects and not the cause. The objective of this study is to show some aspects related to the complications that may appear in this entity when it is associated with pregnancy, the discussion of the clinical problem, the diagnosis and the importance of prevention and timely genetic counseling in this group of patients. We present a black 26 year-old pregnant woman with gestational age of 13.5 weeks, with a personal pathological history of S-? Thalassemia. From a very young age, she presented pain in her limbs, with predominance in the upper right and lower left, fixed, moderate to severe intensity, which does not respond to analgesics, accompanied by pain in hypogastrium that intensifies in the following days. The patient required two blood transfusions. The importance of the preconception visit is reaffirmed to preemptively educate the population before pregnancy to avoid complications(AU)

Ecocardiografia de pacientes talassêmicos sem insuficiência cardíaca em tratamento com transfusões sanguíneas e quelação / Echocardiography in thalassemic patients on blood transfusions and chelation without heart failure

FUNDAMENTO: Pacientes com talassemia major (TM) apresentam hemólise crônica e necessitam de transfusões sanguíneas egularmente que podem causar cardiomiopatia por sobrecarga de ferro e insuficiência cardíaca crônica. A hemocromatose é caracterizada por acúmulo excessivo de ferro nos tecidos; acometimento do coração é a principal causa de óbito em pacientes com talassemia. OBJETIVO: Avaliar as estruturas e a função cardíaca por meio de ecocardiografia com Doppler convencional e Doppler tecidual em pacientes com TM, sem evidência clínica de insuficiência cardíaca. MÉTODOS: Trata-se de estudo observacional prospectivo de 18 pacientes com TM que recebem transfusão sanguínea regularmente. Para avaliar, separadamente, os efeitos da anemia e da transfusão sanguínea, dois grupos controles pareados por gênero, idade, peso e altura foram incluídos: um com indivíduos saudáveis (Saudável, n = 18) e outro com pacientes com anemia por deficiência de ferro (Anemia, n = 18). Análise estatística foi realizada utilizando ANOVA seguida pelo teste de Tukey ou Kruskal-Wallis e teste de Dunn. RESULTADOS: As seguintes variáveis ecocardiográficas apresentaram valores significativamente mais elevados no grupo TM do que nos grupos Anemia e Saudável: índice de volume do átrio esquerdo (Saudável: 16,4 ± 6,08; Anemia: 17,9 ± 7,02; TM: 24,1 ± 8,30 cm/m); razão E/Em septal mitral (Saudável: 6,55 ± 1,60; Anemia: 6,74 ± 0,74; TM: 8,10 ± 1,31) e duração do fluxo reverso em veias pulmonares [Saudável: 74,0 (59,0-74,0); Anemia: 70,5 (67,0-74,0); TM: 111 (87,0-120) ms]. Arazão E/A mitral foi maior no grupo TM do que no grupo Anemia (Saudável: 1,80 ± 0,40; Anemia: 1,80 ± 0,24; TM: 2,03 ± 0,34). Não foram encontradas diferenças entre os grupos em variáveis estruturais do ventrículo esquerdo e em índices de função sistólica. CONCLUSÃO: A ecocardiografia com Doppler convencional e o Doppler tecidual permite que alterações na função diastólica do ventrículo esquerdo sejam identificadas em pacientes assintomáticos com talassemia major.

BACKGROUND: Patients with thalassemia major present chronic hemolysis and require regular blood transfusions which may cause iron overload cardiomyopathy and chronic heart failure. Hemochromatosis is characterized by excessive iron accumulation in tissues, and heart involvement is the main cause of death in patients with thalassemia. OBJECTIVE: The aim of this study was to evaluate cardiac structure and function by conventional Doppler echocardiography and tissue Doppler imaging in patients with TM and no clinical evidence of heart failure. METHODS: This is a prospective observational study including 18 patients with thalassemia major (TM) receiving regular blood transfusion. To separately evaluate anemia and blood transfusion effects, two gender, age, weight, and height-matched control groups were included: one with healthy individuals (Healthy, n=18) and one with iron deficient anemia patients (Anemia, n=18). Statistical analysis was performed using ANOVA followed by Tukey's test or Kruskal-Wallis's and Dunn's test. RESULTS: The following echocardiographic variables presented significantly higher values in TM than the Anemia and Healthy groups: left atrium volume index (Healthy: 16.4±6.08; Anemia: 17.9±7.02; TM: 24.1±8.30 cm³/m²); mitral septal E/Em ratio (Healthy: 6.55±1.60; Anemia: 6.74±0.74; TM: 8.10±1.31); and duration of reverse pulmonary vein flow [Healthy: 74.0 (59.0-74.0); Anemia: 70.5 (67.0-74.0); TM: 111 (87.0-120) ms]. The mitral E/A ratio was higher in TM than Anemia (Healthy: 1.80±0.40; Anemia: 1.80±0.24; TM: 2.03±0.34). No differences were found in left ventricular structures and systolic function indexes. CONCLUSION: Conventional Doppler echocardiography and tissue Doppler allow changes in left ventricular diastolic function to be identified in asymptomatic patients with thalassemia major.

Quality of life among Iranian patients with beta-thalassemia major using the SF-36 questionnaire / Qualidade de vida em pacientes iranianos com beta-talassemia maior usando o questionario SF-36

CONTEXT AND OBJECTIVE Patients with beta-thalassemia major (β-TM) experience physical, psychological and social problems that lead to decreased quality of life (QoL). The aim here was to measure health-related QoL and its determinants among patients with β-TM, using the Short Form-36 (SF-36) questionnaire. DESIGN AND SETTING Cross-sectional study at the Hematology Research Center of Shiraz University of Medical Sciences, in southern Iran. METHODS One hundred and one patients with β-TM were randomly selected. After the participants' demographics and disease characteristics had been recorded, they were asked to fill out the SF-36 questionnaire. The correlations of clinical and demographic factors with the QoL score were evaluated. RESULTS There were 44 men and 57 women of mean age 19.52 ± 4.3 years (range 12-38). On two scales, pain (P = 0.041) and emotional role (P = 0.009), the women showed significantly lower scores than the men. Lower income, poor compliance with iron-chelating therapy and presence of comorbidities were significantly correlated with lower SF-36 scores. These factors were also found to be determinants of worse SF-36 scores in multivariate analysis. CONCLUSIONS We showed that the presence of disease complications, poor compliance with iron-chelating therapy and poor economic status were predictors of worse QoL among patients with β-TM. Prevention and proper management of disease-related complications, increased knowledge among patients regarding the importance of managing comorbidities and greater compliance with iron-chelating therapy, along with psychosocial and financial support, could help these patients to cope better with this chronic disease state. .

CONTEXTO E OBJETIVO Pacientes com beta-talassemia maior (β-TM) vivenciam problemas físicos, psicológicos e sociais que levam à diminuição da qualidade de vida (QV). O objetivo foi determinar a QV relacionada à saúde e seus determinantes em pacientes com β-TM, utilizando questionário SF-36 (Short Form-36). TIPO DE ESTUDO E LOCAL Estudo transversal no Centro de Hematologia e Pesquisa em Universidade de Ciências Médicas de Shiraz, no sul do Irã. MÉTODOS Foram selecionados aleatoriamente 101 pacientes com β-TM. Após registro demográfico e características da doença, eles foram convidados a preencher o questionário SF-36. A correlação entre fatores clínicos e demográficos com escore de QV foi avaliada. RESULTADOS Havia 44 homens e 57 mulheres, com idade média de 19,52 ± 4,3 (variação 12-38) anos. Em duas escalas, dor (P = 0,041) e aspectos emocionais (P = 0,009), as mulheres apresentaram escores significativamente menores aos dos homens. Menor renda, baixa adesão à terapia quelante de ferro e presença de comorbidades foram correlacionadas com escores SF-36 significativamente menores. Esses fatores foram também considerados determinantes de piores escores de SF-36 em análise multivariada. CONCLUSÕES Mostramos que a presença de complicações da doença, a baixa adesão ao tratamento da terapia quelante de ferro e o baixo status econômico são preditores de pior QV em pacientes com β-TM. Prevenção e manejo adequado das complicações relacionadas com a doença, aumento do conhecimento dos pacientes sobre a importância do gerenciamento de comorbidades e ter maior adesão ao tratamento quelante de ferro, considerando também ...

Riesgo de ictus isquémico en niños con Sβ talasemia. Estudio con Doppler transcraneal / Risks of ischemic stroke in children suffering S-thalassemia. Transcranial Doppler study

En niños con anemia por células falciformes, el riesgo de ictus isquémico varía según el genotipo de la hemoglobinopatía. En estos casos, la determinación de la velocidad de flujo sanguíneo mediante Doppler transcraneal (DTC) puede predecir la ocurrencia de isquemia cerebral. Con el objetivo de describir las características del DTC en la Sβ talasemia, se estudiaron 32 enfermos menores de 18 años atendidos en el Instituto de Hematología e Inmunología y remitidos para su evaluación con ultrasonido. La edad media de los niños fue de 10,5 años. Se estudió la misma cantidad de enfermos con cada genotipo Sβ; solamente en un paciente la ventana temporal no fue útil. En el 97 % de los enfermos la velocidad media de flujo en las arterias cerebrales medias era inferior a 170 cm/s y solo en un niño estaban en el rango del grupo condicional. Se registraron asimetrías interhemisféricas en la velocidad media de flujo de las arterias cerebrales anteriores en el 29 por ciento de los casos; en el 19,6 por ciento en las arterias cerebrales medias; y en el 6,4 por ciento en las carótidas internas extracraneales. Los niveles más altos de velocidad media de flujo se registraron en los enfermos con Sβ0 talasemia y en las arterias cerebrales medias. Existió una correlación inversa entre la edad y los niveles de hemoglobina con la velocidad de flujo de los 3 vasos estudiados. El riesgo de ictus isquémico en la Sβ talasemia es bajo, sin diferencia entre los 2 genotipos evaluados

In children with sickle cell anemia, the risk of ischemic stroke varies according to genotype of hemoglobinopathy. In these cases, determining the velocity of blood flow by transcranial Doppler (TCD) can predict the occurrence of cerebral ischemia. In order to describe the characteristics of DTC in Sβ thalassemia, we studied 32 patients younger than 18 treated at the Institute of Hematology and Immunology, who were referred for ultrasound assessment. The age rate of children was 10.5 years old. We studied the same number of patients with each genotype Sβ, in only one patient the time window was not useful. 97 percent of patients the mean flow velocity in the middle cerebral arteries was less than 170 cm/s and only one child was in the range of conditional group. Interhemispheric asymmetries were registered in the average flow rate of the anterior cerebral arteries in 29 percent cases, 19.6 percent registered it in the middle cerebral arteries, and 6.4 percent in the extracranial internal carotid. The highest levels of average flow rate were registered in patients with thalassemia Sβ0 and middle cerebral arteries. There was an inverse correlation between age and hemoglobin levels with the flow rate of three studied vessels. The risk of ischemic stroke in the Sβ thalassemia is low, with no difference between the two assessed genotypes

Intracranial Extramedullary Hematopoiesis in Beta-Thalassemia

Extramedullary hematopoiesis (EMH) represents tumor-like proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. Intracranial EMH is an extremely rare occurrence. Magnetic resonance imaging (MRI) offers a precise diagnosis. It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.

Post-transfusion hypertension, convulsion and intracranial haemorrhage in beta-thalassemia major

The haematologic disorder beta-thalassemia major is common in Pakistan. We describe a patient with undiagnosed thalassemia presenting with hypertension and convulsions and found to have cerebral haemorrhage on neuro-imaging. He had been transfused 2 weeks before this illness. Our experience is similar to a few case reports described in literature that were found to have cerebral haemorrhages post-mortem after a similar clinical presentation. All patients had a blood transfusion within 2 weeks prior to the presentation so association with transfusion has been proposed. We have reviewed the several mechanisms presented and discussed the findings

Transfusion-induced hemoglobinopathy in patients of beta-thalassemia major.

Apparent hemoglobinopathy acquired after blood transfusion is an uncommon cause of diagnostic dilemma resulting in repeated testing and delay in the diagnosis. Out of the 1530 hemoglobin (Hb)- high-performance liquid chromatography (HPLC) performed at our hospital (May 2009 to April 2010), 3 pediatric cases of thalassemia major were detected having posttransfusion hemoglobinopathy with HbS ranging from 9.9% to 18.5%. In all three cases, there was no variant hemoglobin in earlier documented Hb-HPLC. It is important to be aware of and consider apparent transfusion-induced hemoglobinopathy in patients with unusual percentage of variant hemoglobin to avoid unnecessary treatment and counseling.

Chronic myelogenous leukemia in sickle cell/beta0-thalassemia.

Sickle cell/beta 0 -thalassemia (S/β0 -thal) is a compound heterozygous state for βS and β0 thalassemia. There are rare reported cases of patients with sickle cell disease who developed hematological neoplasms including myeloid and lymphoid conditions; however, to the best of our knowledge, chronic myelogenous leukemia (CML) occurring in S/β0 -thal has been reported in one case and this is the second such report.

Salmonella enterica enteritidis arthritis following trauma in a child with thalassemia major.

Osteoarticular infections caused by Non-typhi Salmonella are exceptionally encountered. We report a case of a bacteriologically documented knee joint infection due to Salmonella enterica serotype enteritidis, following trauma in a child with thalassemia major. Emergency arthrotomy combined with antimicrobial therapy was helpful in eradication of infection. Physicians should be aware of this rare manifestation of Non-typhi Salmonella infections in thalassemic patients.

Marcadores eletrocardiográficos para detecção precoce de doença cardíaca em pacientes com talassemia beta maior / Electrocardiographic markers for the early detection of cardiac disease in patients with beta-thalassemia major

OBJETIVO: Analisar comparativamente a dispersão da onda P (DOP) em pacientes com talassemia beta maior (β-TM) e indivíduos saudáveis (controles) para a detecção precoce do risco de arritmias. MÉTODOS: Oitenta e uma crianças com β-TM, com idades entre 4 e 19 anos, e 74 crianças saudáveis (grupo controle) foram submetidas a exame eletrocardiográfico e ecocardiograma transtorácico de rotina para avaliação cardíaca. A DOP foi calculada como a diferença entre as durações máxima e mínima da onda P. RESULTADOS: Houve uma diferença estatisticamente significativa entre o grupo de estudo e o grupo controle no pico de velocidade do fluxo transmitral no início da diástole (E) e na razão E/fluxo transmitral tardio (A). A duração máxima da onda P e a DOP foram significativamente maiores nos pacientes com β-TM do que nos indivíduos controles. CONCLUSÕES: O aumento da DOP em nossos pacientes com β-TM pode estar relacionado à depressão na condução intra-atrial, devido à dilatação atrial, e ao aumento da atividade simpática. Estes pacientes devem ser acompanhados atentamente devido à possibilidade de ocorrência de arritmias com risco de vida.

OBJECTIVE: To comparatively evaluate P-wave dispersion (PWD) in patients with β-thalassemia major (TM) and healthy control subjects for the early prediction of arrhythmia risk. METHODS: Eighty-one children with β-TM, aged 4-19 years, and 74 healthy children (control group) underwent routine electrocardiography and transthoracic echocardiography for cardiac evaluation. PWD was calculated as the difference between the maximum and the minimum P-wave duration. RESULTS: There was a statistically significant difference between study and control groups in peak early (E) mitral inflow velocity and E/late (A) velocity ratio. Maximum P-wave duration and PWD were found to be significantly higher in β-TM patients than in control subjects. CONCLUSIONS: Increased PWD in our β-TM patients might be related to depression of intra-atrial conduction due to atrial dilatation and increased sympathetic activity. These patients should be closely followed up for risk of life-threatening arrhythmias.

Protein C and protein S: Causative factor for developing a hemorrhagic infarct in a HbE/Beta thalassemia child.

A 10-yr-old HbE/Beta thalassemia child who developed subacute to chronic occipitotemporal hemorrhagic infarct with smaller chronic infarct with gliotic changes in the left frontal periventricular white matter. Genetic tests showed that patient was positive for HbE and IVS1-5 mutation and was negative for thrombogenic mutations. Hemorrhagic infarct was confirmed by magnetic resonance imaging study. Antigenic levels of Protein C and Protein S were low. Based on these outcomes, it was concluded that Protein C and Protein S deficiency were the causative factor for developing hemorrhagic infarct in the HbE/ Beta thalassemia patient.

Multiple transfused thalassemia major: Ocular manifestations in a hospital-based population.

Purpose: To study the ocular manifestations in multiple transfused beta-thalassemia major patients and assess the ocular side-effects of iron chelating agents. Materials and Methods: In this prospective observational study, 45 multiple transfused beta-thalassemia major children between six months and 21 years of age were enrolled and assigned groups according to the treatment regimens suggested. Group A received only blood transfusions, Group B blood transfusions with subcutaneous desferrioxamine, Group C blood transfusions with desferrioxamine and oral deferriprone and Group D blood transfusions with deferriprone. Ocular status at the time of enrolment was documented. Subjects were observed quarterly for one year for changes in ocular status arising due to the disease process and due to iron chelation therapy. Children with hemoglobinopathies other than beta-thalassemia major, congenital ocular anomalies and anemia due to other causes were excluded. Results: Ocular involvement was observed in 58% of patients. Lenticular opacities were the most common ocular finding (44%), followed by decreased visual acuity (33%). An increased occurrence of ocular changes was observed with increase of serum ferritin and serum iron levels as well as with higher number of blood transfusions received. Desferrioxamine seemed to have a protective influence on retinal pigment epithelium (RPE) mottling. Occurrence of lenticular opacities and RPE degeneration correlated positively with use of desferrioxamine and deferriprone respectively. Follow-up of patients for one year did not reveal any change in ocular status. Conclusion: Regular ocular examinations can aid in preventing, delaying or ameliorating the ocular complications of thalassemia.

[Beta thalassemia intermedia: about 36 cases]

Thalassemia intermedia empasses a mild clinical and biological spectrum. The aim is to report the clinical and biological features and treatment of this disease. It is a retrospective study about 36 thalassemia intermedia patients [17 males, 19 females]. Epidemiological, haematological aspects and treatment were reported. The diagnosis was carried out at a relatively old age 15 years [1-72].The thalassemia intermedia was characterized by mild facial deformities, splenomegaly and moderate anemia [Hb=9.1 g/dl]. The mean serum ferritin was 518 ng/ml [25-1800].Three phenotypes are caracterised: heterozygosis beta thalassemia, beta° thalassemia and beta° thalassemia. Clinical complications were hypersplenism, extra medullary hematopoiesis, leg ulcers, thrombosis and pulmonary hypertension. Treatment was based on occasionally transfusion and splenectomy on event of hypersplenious [47%]. Evolution of this disease was generally good with a long lifespan at 31 years [6-83]. Thalassemia intermedia is well tolerated. Transfusions and splenectomy were indicated in case of hypersplenious

[Trace back of thalassemic patients with positive HCV markers to their donors in adult thalassemia center]

Transfusion transmitted infection is one of the most important transfusion reactions. In this study, we tried to find new cases of HCV in thalassemic patients having referred to Adult Thalassemia Clinic after 1996 and to trace them back for sources of infection. This was a descriptive study in which all patients were studied; census method of data collection was used. Those patients with no test record before 1996 that appeared to be positive in their first test attempt were not considered a new HCV positive case. The new cases were just those whose past negative HCV Ab test results changed into positive in new test. For data analysis, SPSS version 14 was used. Out of 395 file records, 274[69.4%] were thalassemia major, and 110 [27.8%] intermediate. We had 109 HCV positive cases [27.5%] out of whom 21 were those infected after 1996. Out of the latter, 5 had complete medical records by which 54 blood donors were identified; however, only 37 [68.5%] were traced and found to be negative for HCV Ab. Noteworthy that 81% of these safe donors were shown to be repeated donors. Since there had been one or more donors whose donations had been administered to each patient with no possibility for them to be traced, we were not able to prove the transmission of HCV from donors to recipients. Other possibilities including hospital transmission, patient to patient transmission, and transmission by nurses involved in blood administration should be taken into account

Hematuria in patients with beta-thalassemia major

Our information about renal involvement in beta-thalassemia major is limited. Recently, few studies have reported proteinuria, hypercalcuria, phosphaturia, and oversecretion of tubular damage markers; however, hematuria has not yet been meticulously studied in these patients. We investigated hematuria in patients with beta-thalassemia major. Urinalysis was performed in 500 patients with beta-thalassemia major under a regular blood transfusion program. In those with hematuria [at least 3 to 5 erythrocytes per high-power field] a second urinalysis was done at the next transfusion time. The patients ranged in age from 6 months to 32 years. The male-female ratio was 1.05:1. Hematuria was detected in 55 [10.6%], including 9.8% of those younger than 20 years and 20.0% of those older than 20 years. Hematuria was persistent in 79.2% of the second urinalyses. Sixty-four percent of the patients with hematuria were females. A blood transfusion program had been started during the first year of life in 81% percent of the patients with hematuria. Sterile pyuria was detected in 4% and proteinuria in 16% of the patients with hematuria, while these figures in patients without hematuria were 2.1% [P=.56] and 1.4% [P=.002], respectively. We found that in patients with beta-thalassemia major, the risk of hematuria rises with age. Moreover, proteinuria seems to be more common in those with hematuria. Further studies are needed to ascertain the importance of these findings