Hemangioma esplênico no adulto associado à síndrome de Kasabach-Merritt / Kasabach-Merritt Syndrome-associated adult splenic hemangioma

O hemangioma esplênico, tumor benigno mais frequente do baço, porém, por vezes de difícil diagnóstico, pode integrar a síndrome de Kasabach-Merritt, afecção rara e potencialmente fatal, descrita como associação entre hemangioma, trombocitopenia e coagulopatia de consumo. Apresentamos um caso de paciente de 73 anos em acompanhamento com a hematologia por trombocitopenia crônica a princípio idiopática, que evoluiu com dor abdominal, anemia e derrame pleural. Foi realizada tomografia computadorizada de abdome, que evidenciou massa esplênica sólida heterogênea. A paciente foi submetida a esplenectomia total videolaparoscópica e evoluiu com melhora da dor abdominal e normalização da série plaquetária, sem recorrência do derrame pleural após o procedimento. A raridade e a complexidade do caso, somadas à dificuldade diagnóstica e à abordagem terapêutica, foram fatores que corroboraram para a apresentação desse caso. (AU)

Splenic hemangioma, the most common, but sometimes hard to diagnose, benign tumor of the spleen may integrate Kasabach- Merritt syndrome, a rare and potentially fatal condition described as an association ofhemangioma, thrombocytopenia and consumption coagulopathy. We present a case of a 73-year-old female patient being monitoring, with Hematology due to chronic idiopathic thrombocytopenia who progressed with abdominal pain, anemia and pleural effusion; a computed tomography scan of the abdomen was performed, showing a heterogeneous solid splenic mass. The patient underwent total videolaparoscopic splenectomy and progressed with improvement of abdominal pain and normalization of the platelet series, with no recurrence of pleural effusion after the procedure. The rarity and complexity of the case added to the diagnostic difficulty and therapeutic approach were factors that corroborated for the presentation of this case. (AU)

Síndrome POEMS: um diagnóstico desafiador / POEMS Syndrome: a challenging diagnosis

A síndrome POEMS é um distúrbio multissistêmico. Sua patogênese não está totalmente estabelecida, mas sabe-se que tem relação com fator de crescimento vascular endotelial, interleucinas e fator de necrose tumoral alfa. A idade média de incidência é 50 anos, com maior prevalência em homens. Neuropatia periférica e gamopatia monoclonal estão presentes em todos os pacientes e são consideradas critérios maiores; quando associadas a pelo menos um critério menor, estabelecem diagnóstico da síndrome. As opções de tratamento são radioterapia, corticosteroides e quimioterapia, além de transplante autólogo de células-tronco hematopoiéticas. (AU)

POEMS syndrome is a multisystem disorder. Its pathogenesis isn't fully established, but it is known to be related to endothelial vascular growth factor, interleukins, and tumoral necrosis factor alpha (TNF-α). The mean age at incidence is 50 years, with a higher prevalence in men. Peripheral neuropathy and monoclonal gammopathy are present in all patients, and are considered major criteria; when associated with at least one minor criterium, they establish the diagnosis of the syndrome. Treatment options are radiotherapy, corticosteroids, chemotherapy, as well as autologous hematopoietic stem cell transplantation. (AU)

Gaucher's disease in a patient presenting with hip and abdominal pain

Summary Gaucher's disease is characterized by glucocerebroside accumulation in the cells of the reticuloendothelial system. There are three subtypes. The most common is type 1, known as the non-neuropathic form. Pancytopenia, hepatosplenomegaly and bone lesions occur as a result of glucocerebroside accumulation in the liver, lung, spleen and bone marrow in these patients. Findings associated with liver, spleen or bone involvement may be seen at radiological analysis. Improvement in extraskeletal system findings is seen with enzyme replacement therapy. Support therapy is added in patients developing infection, anemia or pain. We describe a case of hepatosplenomegaly, splenic infarction, splenic nodules and femur fracture determined at radiological imaging in a patient under monitoring due to Gaucher's disease.

Biometria do fígado e do baço em pacientes com lúpus eritematoso sistêmico de início pediátrico / Liver and spleen biometrics in childhood-onset systemic lupus erythematosus patients

RESUMOObjetivoAvaliar as dimensões do fígado e do baço em pacientes com lúpus eritematoso sistêmico de início pediátrico (LESp) e controles saudáveis.MétodosForam submetidos a uma ultrassonografia do abdome 30 pacientes com LESp e 30 voluntários saudáveis controle. Foram feitas duas medições do fígado no lobo hepático esquerdo (craniocaudal e anteroposterior) e três no lobo hepático direito (LHD) (craniocaudal posterior [CCP-LHD], craniocaudal anterior e anteroposterior). Foram também avaliadas três medidas das dimensões do baço: longitudinal, transversal e anteroposterior. Foram avaliados dados demográficos, clínicos e laboratoriais, SLEDAI-2K, ECLAM, SLAM e tratamento.ResultadosA idade média foi semelhante nos pacientes com LESp e controles (170,31 ± 27,81 vs. 164,15 ± 39,25 meses; p = 0,486). A média da dimensão CCP-LHD foi significativamente maior no grupo LESp em comparação com os controles (13,30 ± 1,85 vs. 12,52 ± 0,93, p = 0,044). Não houve diferenças nos outros parâmetros biométricos do fígado e do baço (p > 0,05). Uma análise especifica realizada apenas nos pacientes com LESp de acordo com a dimensão CCP-LHD ≥ 13,3 cm versus < 13,3 cm mostrou que a mediana do SLEDAI-2K [8 (0-18) vs. 2 (0-8), p = 0,004], ECLAM [4 (0-9) vs. 2 (0-5), p = 0,019] e SLAM [5 (1-13) vs. 2 (0-14), p = 0,016] era significativamente maior em pacientes com maior dimensão CCP-LHD, do mesmo modo que a frequência de nefrite (77% vs. 29%, p = 0,010). As enzimas hepáticas foram semelhantes nos dois grupos (p > 0,05). Foi observada uma correlação positiva entre o SLEDAI-2K e a dimensão CCP-LHD (p = 0,001, r = +0,595). Evidenciou-se uma correlação negativa entre a duração da doença e a dimensão longitudinal do baço (p = 0,031, r = −0,394).ConclusãoOs dados levantam a possibilidade de que a atividade da doença pode levar a uma hepatomegalia subclínica e localizada durante o curso da doença. A duração da doença resultou em atrofia do baço em pacientes com LESp.

ABSTRACTObjectiveTo evaluate liver and spleen dimensions in childhood-onset systemic lupus erythematosus (c-SLE) patients and healthy controls.Methods30 c-SLE patients and 30 healthy control volunteers underwent abdominal ultrasound. The following two liver measurements were performed in left hepatic lobe: craniocaudal and anteroposterior and three in right hepatic lobe (RHL): posterior craniocaudal (PCC-RHL), anterior craniocaudal and anteroposterior. Three spleen dimension measurements were also evaluated: longitudinal, transverse and anteroposterior. Demographic, clinical and laboratorial data, SLEDAI-2K, ECLAM, SLAM and treatment were assessed.ResultsMean current age was similar in c-SLE and controls (170.31 ± 27.81 vs. 164.15 ± 39.25months; p = 0.486). The mean of PCC-RHL dimension was significantly higher in c-SLE compared to controls (13.30 ± 1.85 vs. 12.52 ± 0.93, p = 0.044). There were no differences between the other hepatic biometrics and splenic parameters (p > 0.05). Further analysis in c-SLE patients according to PCC-RHL dimension ≥ 13.3 cm versus < 13.3 cm showed that the median of SLEDAI-2K [8(0-18) vs. 2(0-8), p = 0.004], ECLAM [4(0-9) vs. 2(0-5), p = 0.019] and SLAM [5(1-13) vs. 2(0-14), p = 0.016] were significantly higher in patients with higher PCC-RHL dimension, likewise the frequencie of nephritis (77% vs. 29%, p = 0.010). Liver enzymes were similar in both groups (p > 0.05). Positive correlation was observed between SLEDAI-2K and PCC-RHL (p = 0.001, r = +0.595). Negative correlation was evidenced between disease duration and longitudinal dimension of spleen (p = 0.031, r = −0.394).ConclusionOur data raises the possibility that disease activity could lead to a subclinical and localized hepatomegaly during the disease course. Long disease duration resulted to spleen atrophy in c-SLE patients.

Spectrin Tunis (Sp alpha I/78) in a Korean Family with Hereditary Elliptocytosis

No abstract available.

Prenatal Diagnosis of Transient Abnormal Myelopoiesis in a Down Syndrome Fetus

We report a case of transient abnormal myelopoiesis in a Down syndrome fetus diagnosed at 28(+3) weeks of gestation that rapidly progressed to intrauterine death 10 days later. Fetal hepatosplenomegaly with cerebral ventriculomegaly, although not specific, may be a suggestive finding of Down syndrome with transient abnormal myelopoiesis. Prompt fetal blood sampling for liver function test and chromosomal analysis are mandatory for early detection and management.

Ultrasound assessment of normal splenic length and spleen-to-kidney ratio in children

Splenic lengths in 184 normal Jordanian children were measured through the hilum by ultrasound and compared with data from Hong Kong and the United States of America. The spleen to left kidney ratio was calculated to determine whether it was constant and to establish a ratio above which splenomegaly can be diagnosed. Up to age 15 years, little variation in splenic length was observed, but over 15 years splenic length was slightly lower in Jordanian males. Spleen to left kidney ratio was constant at around 1; splenomegaly is highly probable in ratios > / = 1.25

Hepatosplenic B-cell lymphoma associated with hemophagocytic syndrome: a case report

While T-cell non-Hodgkin's lymphoma (NHL) associated with hemophagocytic syndrome (HPS) has been frequently observed, B-cell NHL associated with HPS has been rarely reported. We report a case of hepatosplenic B-cell lymphoma associated with HPS in a 41-year-old woman who presented with fever of unknown origin. An abdominal CT scan revealed splenomegaly with focal splenic infarction. Splenectomy and a liver wedge biopsy showed sinusoidal-pattern infiltration of medium to large tumor cells with positive reaction to a B-lymphocyte marker. Findings on bone marrow examination showed proliferation of histiocytes with avid hemophagocytosis.