Acquired thrombotic thrombocytopenic purpura due to antibody-mediated ADAMTS13 deficiency precipitated by a localized Castleman's disease: a case report.

Acquired ADAMTS13 inhibitor causing thrombotic thrombocytopenic purpura (TTP) may be precipitated by some infections, inflammatory diseases or neoplasia. We reported a case of refractory TTP precipitated by a newly diagnosed localized Castleman's disease (CD). TTP was initially treated with plasma exchange and immunosuppressive therapy with corticosteroids; however the treatment failed to promote sustained response. During hospitalization, an abdominal tumor was diagnosed and resected; the histological analysis revealed a CD of hyaline-vascular variant rich stroma. After tumor removal, the patient achieved a long-lasting clinical remission and normalized ADAMTS13 activity. This clinical case describes a novel association of acquired ADAMTS13 inhibitor and CD. The antibody to ADAMTS13 developed along with the systemic manifestation of CD and promptly disappeared after the resection of the tumor. There are reports of neoplasia-associated thrombotic microangiopathy however direct evidence of CD-dependent ADAMTS13 inhibitor had not yet been reported.

The importance of the clinical classification of adult T-cell leukemia/lymphoma (ATLL) in the prognosis.

BACKGROUND: Adult T-cell leukemia/lymphoma (ATLL), a peripheral T-cell leukemia/lymphoma associated with the human T-cell lymphotropic virus type-1 (HTLV-1), has been classified following the clinical forms defined by Shimoyama in 1991. A suggestion to modify Shimoyama's classification was proposed in 2007 to differentiate within the smoldering patients those who presented nodules or tumors in the skin without lung involvement, which was named the primary cutaneous tumoral (PCT) form of ATLL. In the present study, according to their clinicopathological characteristics, we estimated the mortality rates of 143 ATLL patients from Bahia, Brazil. We also evaluated the importance of classifying PCT/ATLL separately from the smoldering type on disease prognosis. METHODOLOGY/PRINCIPAL FINDINGS: Diagnosis of ATLL was established based on a positive serology for HTLV-1, histopathological and/or cytological diagnosis of peripheral T-cell leukemia/lymphoma. Patients were clinically grouped according to Shimoyama's classification, considering PCT variants separately from the smoldering cases. Bivariate and multivariable survival analyses were applied to identify factors associated with disease prognosis. Significant differences in the median survival time were observed between the clinical types, with the smoldering type presenting the longest median survival (109 months) compared to the other forms (<50 months); the median survival for PCT/ATLL was 20 months. Multivariable analysis confirmed that ATLL clinical types were associated with survival, with a better prognosis for patients with the smoldering and chronic types. Furthermore, skin involvement was related to a worse outcome in the multivariable analysis, regardless of the clinical form and presence of lymphadenopathy. CONCLUSIONS/SIGNIFICANCE: Our results reinforce the importance of considering the PCT/ATLL separately from the smoldering type when classifying ATLL to better define prognosis and treatment, given the significant difference in the survival of patients between the smoldering form and PCT/ATLL. Skin involvement should also be considered an independent prognostic factor in patients with ATLL.

Evaluation of anemia after long-term treatment with imatinib in chronic myeloid leukemia patients in chronic phase.

INTRODUCTION: The incidence of grade 3-4 anemia was reported to be 3% with imatinib therapy for newly diagnosed chronic myeloid leukemia (CML) in the chronic phase (CP). However, there are few data regarding the causes and the development of anemia after long-term treatment. This study aimed to evaluate the incidence of anemia after at least two years of imatinib treatment of CML patients in the CP and to identify other contributing causes of anemia in this population. PATIENTS AND METHODS: We performed a retrospective analysis of 97 CML patients in the CP treated with imatinib for at least two years. We analyzed the hemoglobin (Hb) levels of CML patients at diagnosis, upon initiation of treatment with imatinib and after two years of imatinib treatment, and investigated other causes of anemia in this population. RESULTS: Most of the patients presented Hb levels below the normal range (80.4%) after the second year of treatment, 17.9% grade 2 and 1.3% grade 3. In 13 cases (16.7%), anemia was attributed to resistance and in 13 cases (16.7%) the following causes were identified: iron deficiency (n=5), hypothyroidism (n=2), vitamin B12 deficiency (n=3), acquired immune deficiency syndrome (AIDS) (n=1), pulmonary tuberculosis (n=1) and renal toxicity (n=1). In 52 patients (66.6%), there were no other factors contributing to anemia, except imatinib treatment. CONCLUSION: Regular follow-up is required to identify the causes of anemia not related to CML or imatinib toxicity. The importance of investigating secondary causes of anemia should be emphasized, especially in patients with good adherence to treatment and satisfactory therapeutic response.

Evaluation of anemia after long-term treatment with imatinib in chronic myeloid leukemia patients in chronic phase

ABSTRACT Introduction: The incidence of grade 3-4 anemia was reported to be 3% with imatinib therapy for newly diagnosed chronic myeloid leukemia (CML) in the chronic phase (CP). However, there are few data regarding the causes and the development of anemia after long-term treatment. This study aimed to evaluate the incidence of anemia after at least two years of imatinib treatment of CML patients in the CP and to identify other contributing causes of anemia in this population. Patients and methods: We performed a retrospective analysis of 97 CML patients in the CP treated with imatinib for at least two years. We analyzed the hemoglobin (Hb) levels of CML patients at diagnosis, upon initiation of treatment with imatinib and after two years of imatinib treatment, and investigated other causes of anemia in this population. Results: Most of the patients presented Hb levels below the normal range (80.4%) after the second year of treatment, 17.9% grade 2 and 1.3% grade 3. In 13 cases (16.7%), anemia was attributed to resistance and in 13 cases (16.7%) the following causes were identified: iron deficiency (n = 5), hypothyroidism (n = 2), vitamin B12 deficiency (n = 3), acquired immune deficiency syndrome (AIDS) (n = 1), pulmonary tuberculosis (n = 1) and renal toxicity (n = 1). In 52 patients (66.6%), there were no other factors contributing to anemia, except imatinib treatment. Conclusion: Regular follow-up is required to identify the causes of anemia not related to CML or imatinib toxicity. The importance of investigating secondary causes of anemia should be emphasized, especially in patients with good adherence to treatment and satisfactory therapeutic response.

Evaluation of extracurricular internships in the adult's intensive care units. / Avaliação dos estágios extracurriculares de medicina em unidade de terapia intensiva adulto.

OBJECTIVES: Students of Salvador - BA, Brazil were trained in critical care medicine by accomplishing extracurricular internships. This study aims to detect changes in attitude and interest of students who concluded these internships as well as the most frequent activities developed. METHODS: Descriptive cross-sectional survey conducted with students who did extracurricular internships in adult intensive care units during the second semester of 2006. A self-administered questionnaire was given using objective questions. RESULTS: We evaluated 49 students. Interest in becoming an intensivist was classified as high/very high by 32.7% before internship, after which 61.2% reported increased interest. Before internship, students on a 1 to 5 scale rated the importance of critical care medicine as 4.55 ± 0.70. After internship, 98% felt more confident to refer a patient to the intensive care unit, 95.9% to evaluate with supervision, patients admitted to intensive care units and 89.8% to attend patients in the emergency room. The most common procedures observed were: central venous access (100%), peripheral venous access (91.8%) and orotracheal intubation (91.8%). Topics ranked in terms of interest from 1 to 5 were: systemic inflammatory response syndrome/sepsis (4.82 ± 0.48), shock (4.81 ± 0.44) and cardiopulmonary resuscitation (4.77 ± 0.55). CONCLUSIONS: This study showed that internships in adult intensive care units of Salvador (BA), Brazil provided students with greater assurance to evaluate critical patients, increased their interest to follow an intensivist physician career and allowed contact with the main procedures and topics related to critical care medicine.

Avaliação dos estágios extracurriculares de medicina em unidade de terapia intensiva adulto / Evaluation of extracurricular internships in the adult's intensive care units

OBJETIVOS: A formação em medicina intensiva pelos estudantes de Salvador (BA) tem acontecido através de estágios extracurriculares. Este estudo visou detectar mudanças na postura e no interesse dos acadêmicos que concluíram estes estágios e os tipos mais comuns de atividades desenvolvidas. MÉTODOS: Estudo transversal descritivo realizado com estudantes que fizeram estágios extracurriculares em unidades de terapia intensiva adulto no segundo semestre de 2006. Utilizou-se um questionário auto-aplicável com questões objetivas. RESULTADOS: Foram entrevistados 49 estudantes. O interesse em se tornar intensivista foi classificado como alto/muito alto por 32,7 por cento antes do estágio, ao final 61,2 por cento referiram aumento do interesse. A média de 1 a 5, sobre a importância da medicina intensiva para o acadêmico atualmente foi de 4,55±0,70. Após o estágio 98 por cento sentem-se mais seguros em indicar um paciente para unidades de terapia intensiva e 95,9 por cento em avaliar, sob supervisão, os pacientes internados em unidades de terapia intensiva e 89,8 por cento em atender pacientes nas emergências. Os procedimentos mais observados foram: acesso venoso central (100 por cento), acesso venoso periférico (91,8 por cento) e a intubação orotraqueal (91,8 por cento). Numa escala de 1 a 5, os tópicos classificados como de maior interesse foram: síndrome de resposta inflamatória sistêmica e sepse (4,82±0,48), choque (4,81 ± 0,44) e reanimação cardiopulmonar (4,77 ± 0,55). CONCLUSÕES: O presente estudo mostrou que os estágios extracurriculares em unidades de terapia intensiva adulto de Salvador (BA) fornecem ao estudante maior segurança em avaliar pacientes graves, aumenta o interesse do mesmo pela carreira de intensivista e permite o contato com os principais procedimentos e tópicos relacionados à MI no dia-a-dia das unidades de terapia intensiva.

OBJECTIVES: Students of Salvador - BA, Brazil were trained in critical care medicine by accomplishing extracurricular internships. This study aims to detect changes in attitude and interest of students who concluded these internships as well as the most frequent activities developed. METHODS: Descriptive cross-sectional survey conducted with students who did extracurricular internships in adult intensive care units during the second semester of 2006. A self-administered questionnaire was given using objective questions. RESULTS: We evaluated 49 students. Interest in becoming an intensivist was classified as high/very high by 32.7 percent before internship, after which 61.2 percent reported increased interest. Before internship, students on a 1 to 5 scale rated the importance of critical care medicine as 4.55 ± 0.70. After internship, 98 percent felt more confident to refer a patient to the intensive care unit, 95.9 percent to evaluate with supervision, patients admitted to intensive care units and 89.8 percent to attend patients in the emergency room. The most common procedures observed were: central venous access (100 percent), peripheral venous access (91.8 percent) and orotracheal intubation (91.8 percent). Topics ranked in terms of interest from 1 to 5 were: systemic inflammatory response syndrome/sepsis (4.82 ± 0.48), shock (4.81 ± 0.44) and cardiopulmonary resuscitation (4.77 ± 0.55). CONCLUSIONS: This study showed that internships in adult intensive care units of Salvador (BA), Brazil provided students with greater assurance to evaluate critical patients, increased their interest to follow an intensivist physician career and allowed contact with the main procedures and topics related to critical care medicine.