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)

Histopathological and immunophenotypical assessment of canine primary splenic lymphoma according to the World Health Organization / Avaliação histopatológica e imunofenotípica segundo a classificação da Organização Mundial da Saúde do linfoma esplênico primário em cães

Although there are several studies addressing multicentric lymphoma in dogs, data regarding splenic lymphoma remains scarce. The diagnosis of splenic lymphoma using the World Health Organization (WHO) classification system can aid prognostic characterization of splenic lymphoma. The aim of this study was to evaluate the most common histological types of splenic lymphoma in dogs from Brazil according to the WHO classification. We assessed 33 cases of splenic lymphoma diagnosed by histopathologic and immunohistochemical (IHC) analysis submitted to VETPAT- Pathology Laboratory, Campinas-SP, Brazil. IHC was performed using antibodies against CD3 for T-cell and CD79α for B-cell identification . Mean age of patients with splenic lymphoma was 9.8 years. The most affected breeds were mixed breed dogs (33%) followed by Pit bulls and Yorkshires (9.0%). The most prevalent histological type was marginal zone B-cell lymphoma (60.7%) followed by diffuse large B-cell lymphoma (12.1%) and lymphoblastic T-cell lymphoma (12.1%). Histological and immunohistochemical characterization of splenic lymphoma is important due to the high prevalence of indolent lymphomas such as marginal zone, which may be less aggressive and thus have different prognostic and distinct forms of treatment when compared to high-grade lymphomas.(AU)

Embora existam diversos estudos a respeito do linfoma multicêntrico em cães, os dados sobre linfoma esplênico primário são escassos. O diagnóstico do linfoma esplênico utilizando a classificação da Organização Mundial da Saúde (OMS) pode melhorar a caracterização da doença. O objetivo do estudo foi avaliar os principais tipos de linfoma esplênico primário em cães no Brasil de acordo com a classificação da OMS. Foram avaliados 33 casos de linfoma esplênico diagnosticados por histopatologia e imuno-histoquímica submetidos ao Laboratório de Patologia Veterinária (VETPAT, Campinas/SP). A imuno-histoquímica foi realizada utilizando os anticorpos CD3 para linfomas T, CD79α para linfomas B. A média de idade dos pacientes com linfoma esplênico foi de 9,8 anos. Os animais sem raça definida (SRD) foram os mais acometidos (33%) seguidos de PitBulls e Yorkshire (9,0%). O tipo histológico mais comum foi o linfoma de zona marginal representando 60,7% dos casos seguido do linfoma difuso de grandes células B (12,1%) e linfoma linfoblástico T (12,1%). A caracterização histopatológica e imuno-histoquímica do linfoma esplênico é importante devido à alta prevalência de linfomas indolentes como o linfoma de zona marginal, que devido ao seu comportamento indolente apresenta prognóstico e tratamento distintos quando comparado aos linfomas de alto grau.(AU)

Enfermedad de hígado poliquístico asociado con quiste esplénico / Polycystic liver disease associated with splenic cyst

La enfermedad poliquística del hígado en el adulto es una condición infrecuente y benigna, autosómica dominante, asociada con frecuencia a enfermedad poliquística renal. Se caracteriza por la formación de quistes hepáticos numerosos, con o sin afectación renal. Algunos otros factores probablemente implicados en su génesis son la edad, sexo femenino, embarazo, y el uso de anticonceptivos orales. Las terapias quirúrgicas incluyen la aspiración percutánea del quiste con el escleroterapia, fenestración laparoscópica, fenestración laparotómica, la resección del hígado y el trasplante del hígado. Caso: Hombre de 50 años de edad con diagnóstico de hígado poliquístico asociado a un quiste esplénico diagnosticado por TAC abdominal y que se trató con una técnica de videoloparoscopia. Conclusión: La intervención quirúrgica para la enfermedad del hígado poliquístico sintomático se ha asociado a una morbilidad significativa. La fenestración videolaparoscópica de los quistes es un método seguro y extremadamente efectivo y se debe considerar como tratamiento inicial para la enfermedad del hígado poliquístico.

Polycystic liver in the adult is a rare and benign, autosomal dominant disorder, frequently associated with renal polycystic disease. It is distinguished by formation of numerous hepatic cysts, with or without renal involvement. Some other factors are also thought to be involved in its genesis, such as age, female gender, pregnancy, and the use of oral birth control pills. Surgical therapies include percutaneous cyst aspiration with sclerotherapy, laparoscopic fenestration, open fenestration, liver resection and fenestration and liver transplantation. Case: Polycystic liver disease associated with splenic cyst was diagnosed in a 50 years old man by abdominal computed tomography imaging and videolaparoscopic finding. Conclusion: Surgical intervention for symptomatic polycystic liver disease has been associated with significant morbidity. Laparoscopic cysts fenestration was safe and extremely effective and should be considered as initial treatment for polycystic liver disease.

Non-gastric Marginal Zone B-cell Lymphoma in Korea: Clinical Features, Treatment, and Prognostic Factors

Marginal zone B-cell lymphoma (MZL) is the second most common subtype of non-Hodgkin's lymphoma in Korea (17.3%). Mucosa-associated lymphoid tissue (MALT) can develop in almost any organ as a result of exposure to a persistent stimulus, such as chronic infection or certain autoimmune processes. Under conditions of prolonged lymphoid proliferation, a malignant clone may emerge, which is followed by the development of a MALT lymphoma. Whereas MALT lymphoma of the stomach is the most common and the most extensively studied, we focus on non-gastric MZL studies conducted in Korea that highlight the most recent advances with respect to MZL definition, etiology, clinical characteristics, natural history, treatment approaches, outcomes, and prognostic factors. Moreover, we discuss current organ-specific considerations and controversies, and identify areas for future research.

Linfangioma esplénico gigante: Caso clínico / Gigantic cystic splenic lymphangioma: Report of one case

Splenic lymphangiomas are benign primary simple or multiple tumors. They are incidentally diagnosed in children and people younger than 20 year-old. We report a 26-year-old female presenting with a history of five months of abdominal pain localized over the left upper hemi abdomen. A computed tomographic abdominal scan demonstrated a cystic tumor of 16 cm diameter originating from the spleen. Hydatic serology was negative and all blood tests were normal. At surgery, a spienic cyst was found and almost all the cystic wall was excised, leaving only a small portion attached to the spleen. The pathological report described spienic parenchyma with endothelial lining over the internal side of the cyst. Immunohystochemical stains of the endothelial cells were positive for VIII Factor and D2-40 that are specific for lymphatic endothelium, yielding the final diagnosis of a cystic spienic lymphangioma.

Malignant lymphoma without lymphadenopathy.

Hepatosplenic T-Cell lymphoma (HSTCL) is a rare form of extra-nodal post-thymic T-cell non-Hodgkin's lymphoma that primarily involves liver and spleen with B symptoms, with a characteristic absence of lymphadenopathy. We report such an entity in a 65-year-old man who was diagnosed to have multiple myeloma and treated for the same for two years. A clinical diagnosis of secondary myelofibrosis was suspected and was investigated, when he developed pancytopenia and massive hepatosplenomegaly at one of his follow-up visits. The patient underwent therapeutic splenectomy with a simultaneous wedge biopsy of the liver and with their corresponding histopathological and immunohistochemical features, the diagnosis of HSTCL was clinched.

Splenic infarct as a diagnostic pitfall in radiology.

Follow-up of colorectal carcinoma after therapy is based on symptoms, tumor markers, and imaging studies. Clinicians sometimes face diagnostic dilemmas because of unusual presentations on the imaging modalities coupled with rising serum markers. We report a case of colorectal carcinoma that presented with gastrointestinal symptoms 14 months after completion of treatment. Investigations showed rise in carcinoembryonic antigen (CEA). Suspecting disease recurrence, complete radioimaging workup was performed; the only abnormality detected was a smooth, hypodense area in the posterior third of the spleen on contrast-enhanced computed tomography abdomen. In view of the previous diagnosis of carcinoma colon, the symptoms reported by the patient, the elevated CEA, and the atypical CECT appearance, a diagnosis of splenic metastasis was made. The patient was subjected to splenectomy as a curative treatment. However, the histopathological report revealed it to be a splenic infarct. The present case reemphasizes the limitations of radiological studies in the follow-up of carcinoma colon.

Splenic lymphoma with villous lymphocytes.

Splenic lymphoma with villous lymphocytes (SLVL) is a rare disorder that comprises less than 1% of lymphoid neoplasms. It is the leukemic counterpart of splenic marginal zone lymphoma (SMZL) and is characterized by splenomegaly, often with no lymphadenopathy, moderate lymphocytosis and villous lymphocytes on peripheral blood smear. Here, we report a case of SLVL in a 56-year-old male with very high leukocyte counts, massive splenomegaly and relatively few leukemic cells with subtle villous projections on the surface. This disorder is often confused with other chronic lymphoproliferative disorders, especially chronic lymphocytic leukemia (CLL) and hairy cell leukemia and should be differentiated from them. We are reporting this case to highlight the diagnostic pitfalls associated with this disorder.

Splenic tumor presenting as pyrexia of unknown origin.

Pyrexia of unknown origin has always been a challenging problem to diagnose for physicians. Here we present a case of a splenic tumor, which after histopathology and immunohistochemistry, two possibilities were considered, a diffuse large cell lymphoma--plasmablastic variant and second an anaplastic plasmacytoma. The patient was treated with chemotherapy and on followup he has no evidence of recurrence or any residual lesion.

Nódulos fibro-histiocíticos no baço de uma cadela: relato de caso / Splenic fibroistiocytic nodules in a bitch: case report

Descreve-se a ocorrência de múltiplos nódulos fibro-histiocíticos no baço de uma cadela Rottweiler, de sete anos. O diagnóstico foi feito por histopatologia e confirmado por imunoistoquímica positiva para ED1, CD18, vimentina e lisozima. O animal foi sacrificado três meses após o diagnóstico, por severo agravamento dos sinais clínicos.

Multiple fibrohistiocytic nodules in the spleen of a 7-year-old female Rottweiler were reported. The diagnosis was made by histology and immunohistochemistry; cells were positive for ED1, CD18, vimentin and lysozyme. The dog was euthanatized three months after the tumor was diagnosed, due to severe clinical signs.

Primary splenic lymphoma

A middle-aged lady presented with fever and splenomegaly and had been provisionally treated for malaria, typhoid and tuberculosis. Diagnostic splenectomy was performed which revealed diffuse large cell lymphoma, B type, localized to spleen. Patient had remission of disease after splenectomy

A Case of Splenic Pseudocyst Difficult to Differentiate from Splenic Lymphangioma / 대한소화기학회지

Splenic pseudocyst is a rare complication of abdominal trauma. Although it is rare, splenic pseudocyst is well-documented in the literature. According to the current classification, approximately 30% of all splenic cysts or pseudocysts result from direct abdominal trauma. In addition, chronic pancreatitis leads to change of nearby organs with possible acute and chronic complications including splenic lesions. This unusual complication can occur in both emergent and nonemergent conditions. The useful diagnostic procedures to assess intrasplenic pseudocyst are sonogram, CT scan, splenic scan, and occasionally angiography. However, definite diagnosis of pseudocyst is possible only after splenectomy when the absence of epithelial lining is confirmed histologically. Splenic pseudocyst requires surgical resection. We experienced a 31-year-old man who confirmed of warmness in the left side of back with left upper quadrant abdominal pain for several months. First impression was splenic lymphangioma based on CT scan and sonogram finding. Splenectomy was performed. Microscopic examination revealed splenic pseudocyst with fibrous capsule without epithelial lining.

Cystic lymphangioma of the spleen--a case report.

Splenic cystic lymphangioma is a very rare condition. It occurs commonly in children in whom it is often an incidental imaging finding. In the absence of histologic confirmation, it usually mimics hydatid disease and other cystic vascular proliferations of the spleen. We present a case of cystic lymphangioma of the spleen in 15 years old female.

Three Cases of Diffuse Large B-Cell Lymphoma Presenting as Primary Splenic Lymphoma

Primary splenic lymphoma (PSL) is often defined as generalized lymphoma with splenic involvement as the dominant feature. It is a rare disease that comprises approximately 1% of all malignant lymphomas. We investigated three cases of non-Hodgkin's splenic lymphoma that had different clinical features on presentation. The patients' survival times from diagnosis ranged from 59 to 143 months, without evidence of relapse after splenectomy and chemotherapy, with or without radiotherapy. This data suggest that PSL is potentially curable. Further studies are needed to evaluate the impact that different treatment modalities without splenectomy have on patient survival.

Simultaneous occurrence of follicular lymphoma and mixed-cellularity Hodgkin's lymphoma: lymph node and extranodal involvement

Os autores apresentam um caso raro e bem caracterizado de linfoma composto no baço e linfonodos. A ocorrência simultânea de um linfoma de Hodgkin e um linfoma não-Hodgkin foi demonstrada em um homem de 66 anos admitido com anemia, hepatosplenomegalia e múltiplos linfonodos abdominais. O estudo morfológico do baço e linfonodo do hilo esplênico mostrou infiltração por duas neoplasias distintas. O fígado estava envolvido por linfoma não-Hodgkin e o linfonodo peripancreático mostrava um linfoma de Hodgkin. As células de Reed-Sternberg (RS) expressaram CD 15 e CD 30, enquanto as células do linfoma não-Hodgkin apresentaram os aspectos imunoistoquímicos clássicos do linfoma folicular. Na literatura médica, este é o quinto caso descrito de envolvimento do baço por linfoma composto. A incidência, aspectos clínicos e imunoistoquímicos desta rara associação são discutidos.

Splenic haemangioma and Kasabach-Merritt Syndrome as differential diagnosis for abdominal mass in a newborn: case report

Apresentamos um caso raro de hemangioma esplênico em um recém-nascido do sexo feminino, apresentando-se como massa abdominal, coagulopatia e trombocitopenia. No ato operatório observou-se uma massa tumoral vascular do pólo inferior do baço. A paciente encontra-se em acompanhamento ambulatorial. O diagnóstico e as opções de tratamento foram revistas e discutidas. Os autores revisaram a literatura sobre hemangioma esplênico em recém-nascidos e observaram ser este o terceiro caso de associação entre hemangioma esplênico e Síndrome de Kasabach-Merritt. O hemangioma esplênico é uma doença rara no diagnóstico diferencial das massas abdominais em recém-nascidos. O hemangioma é a neoplasia benigna mais freqüente do baço. A anemia, a trombocitopenia e a coagulopatia são vistos com freqüência em hemangiomas cavernosos grandes associados à Síndrome de Kasabach-Merritt (KMS). O hemangioma cavernoso esplênico associado com esta síndrome é extremamente raro.