Pancitopenia em pacientes com anemia perniciosa. Estudo descritivo de 33 casos. / Pancytopenia in patients with pernicious anemia. Descriptive study of 33 cases.

Objetivo: Discriminar as variáveis de sexo e idade nos pacientes com anemia perniciosa; estudar seu perfil hematimétrico; verificar a prevalência de outras doenças autoimunes e anemia perniciosa; analisar a incidência da pancitopenia e sua relação com alterações laboratoriais comum na doença; e avaliar a frequência dos autoanticorpos anticélulas parietais e antifator intrínseco. Métodos: Estudo transversal descritivo, de base clínica e laboratorial, de 33 prontuários de pacientes com anemia perniciosa, diagnosticados em um ambulatório de hospital terciário de atenção à saúde, no período de junho de 2009 a junho de 2014. Para analisar a relação da presença e da ausência de pancitopenia com os níveis da enzima lactato desidrogenase e vitamina B12, foi utilizado o teste qui quadrado. O programa utilizado foi o software Epi Info, versão 7. Resultados: Na amostra, 63,6% eram mulheres, e a idade média geral foi de 47,3 anos. Doenças autoimunes estavam associadas em 30,3% dos pacientes. A pancitopenia esteve presente em 39,4% dos pacientes. Houve significância estatística na relação da pancitopenia com os níveis de enzima lactato desidrogenase (p<0,05). A prevalência do antifator intrínseco foi de 69,7% e dos autoanticorpos anticélulas parietais foi de 72,7%. Conclusão: A pancitopenia mostrou-se um achado significante na população com anemia perniciosa, assim como níveis elevado de LDH, acrescentando a anemia perniciosa como um diagnóstico diferencial de tais alterações laboratoriais.(AU)

Objective: To discriminate the gender and age variables in patients with pernicious anemia; to study erythrocyte profile; to check the prevalence of other autoimmune diseases and pernicious anemia; to analyze the incidence of pancytopenia and its relationship with common laboratory abnormalities in the disease; to evaluate the frequency of anti-gastric parietal cell antibodies, and anti-intrinsic factor antibodies. Methods: Descriptive, cross-sectional study of clinical and laboratorybased medical records of 33 patients with pernicious anemia diagnosed in an outpatient's department of a tertiary healthcare center, in the period between June 2009 and June 2014. To analyze the relationship between the presence and absence of pancytopenia with levels of lactate dehydrogenase enzyme and levels of Vitamin B12 we used the chi-squared test. The software used was Epininfo version 7. Results: The sample showed 63.6% women and 36.4% men with a mean age of 47.3 years. Autoimmune diseases were associated in 30.3% of the patients. Pancytopenia was present in 39.4% of patients. There was statistically significant relationship of pancytopenia with lactate dehydrogenase enzyme levels (p <0.05). The frequency of anti-intrinsic factor antibodies was 69.7%, and the antigastric parietal cell antibodies was 72.7%. Conclusion: Pancytopenia proved to be a significant finding among the population with pernicious anemia and high levels of LDH, which includes pernicious anemia as a differential diagnosis of such laboratory alterations.(AU)

Bone Marrow Suppression and Hemophagocytic Histiocytes Are Common Findings in Korean Severe Fever with Thrombocytopenia Syndrome Patients

The causes of cytopenia in patients with severe fever with thrombocytopenia syndrome (SFTS) are not fully understood until now. We reviewed the bone marrow (BM) findings of patients with SFTS to unravel the cause of the cytopenia. Three Korean SFTS were enrolled in this study. Thrombocytopenia, neutropenia, and anemia were detected in all three patients. Severe hypocellular marrow (overall cellularity <5%) and a decreased number of megakaryocytes were noted in one patient, and hypo-/normocellular marrow and an increased number of hemophagocytic histiocytes were observed in two patients. Megakaryocytes were relatively preserved in two patients. Although a limited number of cases are available, our observations suggest that both BM suppression and peripheral destruction or sequestration are causes of cytopenia of patients with SFTS. To the best of our knowledge, this is the first well documented pathologic evaluation of Korean SFTS.

A Rare Case of Acute Lymphoblastic Leukemia with t(12;17)(p13;q21) / 대한진단검사의학회지

Patients with ALL rarely present with t(12;17)(p13;q21) as the primary clonal abnormality; this abnormality is associated with the expression of myeloid antigens. In this study, we have reported presumably the first case of this chromosomal abnormality in Korea, thereby facilitating the delineation of a distinct subtype of ALL. A 57-yr-old woman was referred to our hospital because of pancytopenia. Peripheral blood examination showed 55% blasts. The bone marrow was markedly hypercellular, and about 82.4% of all nucleated cells were blasts. The results of immunophenotyping and cytochemical staining suggested early precursor B-ALL. Cytogenetic analysis of the bone marrow cells showed a complex karyotype, including a reciprocal translocation between the short arm of chromosome 12 and the long arm of chromosome 17, t(12;17)(p13;q21). Data from array comparative genomic hybridization were almost consistent with the cytogenetic findings.

Pancytopenia in a child associated with hepatitis A infection.

Pancytopenia is a very rare condition associated with hepatitis A infection. We managed a 12 year old boy who had hepatitis A infection with anemia. His hemogram and bone marrow examination were suggestive of pancytopenia. Pancytopenia recovered without any specific therapy. There are case reports of severe aplastic anemia with hepatitis A infection that required immunosuppressive therapy. The present case did not require any aggressive therapy and recovered. In a young child with hepatitis A infection and anemia, bone marrow depression should be suspected. The pancytopenia may be transient as exemplified by the present case.

Amibas de vida libre y anemia aplástica grave en niños / Aplastic anemia and free-living amoeba in two children

Introducción. Las amibas de vida libre habitualmente viven como fagótrofas en el agua y suelo donde se alimentan de bacterias; también pueden producir infecciones del sistema nervioso central y otros tejidos en seres humanos y animales. La anemia aplástica es una entidad caracterizada por pancitopenia secundaria a disminución de la producción en médula ósea de todos los elementos formes de la sangre y ausencia de enfermedad primaria que infiltre, reemplace o anule la hematopoyesis activa. Casos clínicos. Se presentan 2 niños con historia de sangrados, pancitopenia y el diagnóstico de anemia aplástica grave por biopsia y aspirado de médula ósea, que finalmente fallecieron. Se muestran los hallazgos de amibas de vida libre en la médula. Conclusión. En estos casos no se encontró agente causal de la anemia aplástica, y se sugiere a las amibas como oportunistas del padecimiento.

Bone marrow necrosis in paediatric patients.

Necrosis of bone marrow is a very rare condition with a prevalence ranging from 0.15 to 7 percent. It is mostly not compatible with life. We diagnosed four cases of necrotic marrow out of 830 bone marrow aspirations done in last five years of time. All were paediatric patients having peripheral pancytopenia. Three out of four patients expired within one month of diagnosis.

Tratamento do hiperesplenismo por embolizaçäo esplênica em uma paciente com câncer de ovário para possibilitar a administraçäo de quimioterapia antineoplásica / Treatment of hyperplenism in patient with ovarian cancer to allow chemotherapy

O hiperesplenismo é uma síndrome caracterizada por esplenomegalia que, por sua vez, produz um quadro de anemia, leucopenia, trombocitpenia ou qualquer combinaçäo destas citpenias no contexto de uma medula óssea normal ou hiperplásica. Estas citopenias, por definiçäo, se corrigem com a esplenectomia. o quadro de leucopenia, anemia e/ou trombocitopenia decorrentes do hiperesplenismo é especialmente preocupante em pacientes oncológicos. Nestes pacientes, o hiperesplenismo muitas vezes se constitui em um fator limitante à administraçäo de doses terapêuticas de drogas quimioterápicas mielotóxicas. Apresentamos aqui um caso de uma paciente de 61 anos com esplenomegalia à doença de Caroli e portadora de cistoadenocarcinoma seroso papilífero invasivo estádio III (FIGO), cuja quimioterapia foi complicada por pancitopenia prolongada. O mielograma revelou uma medula óssea hipercelular sem evidência de alteraçöes mielodisplásicas ou megaloblásticas compatível com a presença de hiperesplenismo. Com a condiçäo clínica precária desta paciente precluia a esplenectomia, procedeu-se à embolizaçäo parcial esplênica (EPE) que resultou em melhora importante e prolongada dos seus níveis hematimétricos. Pudemos, assim, administrar quimioterapia antineoplásica para esta paciente sem a ocorrência de mielosupressäo importante. Concluímos, portanto, que a embolizaçäo esplênica é uma alternativa para casos de pacientes com câncer e necessidade de quimioterapia e com pancitopenia intensificada pela presença de hiperesplenismo.

Lúpus eritematoso sistêmico na infância, tendo como apresentaçäo febre de origem obscura, pancitopenia e esplenomegalia: relato de caso / Lupus erythematosus systemic in childhood, to be found as fever of obscure origin, pancytopenia and splenomegaly

Relata-se um caso de Lúpus Eritematoso Sistêmico que teve como apresentaçäo febre de origem obscura, pancitopenia e esplenomegalia em criança do sexo feminino (11 anos), sem queixas relacionadas ao aparelho osteoarticular, que só foi diagnosticado após quatro meses de acompanhamento clínico-laboratorial, um episódio de alopécia e persistência na conduta expectante, apesar das pressöes exercidas pelos pais na determinaçäo imediata do tratamento

Angioimmunoblastic lymphadenopathy with dysproteinemia complicated by pancytopenia

Angioimmunoblastic lymphadenopathy with dysproteinemia [AILD] is described. The patient had generalised lymphadenopathy, skin rash and hepatosplenomegaly. Serum IgA level was raised. Lymphnode biopsy showed effacement of its architecture, infiltration by numerous immunoblast and plasma cells and abundant proliferation of arborising small blood vessels. After initial improvement on steroid, the patients 's general condition deteriorated and he developed pancytopenia and died

Fatal pancytopenia in falciparum malaria.

Various pernicious syndromes in Plasmodium falciparum infection are being reported with increasing frequency from tropical countries. A rare case of fatal pancytopenia associated with falciparum malaria is described. The patient developed fulminant aspiration bronchopneumonia which was unresponsive to antibiotic therapy and contributed to the development of adult respiratory distress syndrome. He also had severe uncontrolled gastrointestinal bleeding and possibly an intracerebral haemorrhage. Anaemia and thrombocytopenia are well known in malaria but severe leucopenia is very rare and pancytopenia has not been reported.