Extramedullary hematopoiesis (EMH) generally occurs in patients with deficient bone marrow hematopoiesis secondary to either peripheral red cell destruction or marrow replacement. EMH is most commonly seen in the liver and spleen as a diffuse lesion. Rarely EMH presents as a solitary mass, posing a diagnostic dilemma. In asymptomatic patients without obvious evidence of hemato-pathology, the differential diagnosis is even more complex. Despite several reports describing the radiographic findings in EMH, fewer promote the use of fine needle aspiration (FNA) in making this diagnosis.
Primary effusion lymphoma (PEL) is an unusual class of non-Hodgkin's lymphoma that develops in body cavities, without associated mass lesions. It has been linked to human herpes virus 8 (HHV-8), an etiological factor of Kaposi's sarcoma. Although PEL is a B-cell lymphoma, the neoplastic cells are usually of the "null" phenotype by immunocytochemistry. The relative infrequency of this entity, the absence of wide casuistic allowing a better characterization, and its unfavorable outcome, strongly support the need of a deeper knowledge. We report the clinico-biological findings of a 49-year-old male who was iatrogenically suppressed patient for 29 years because of renal transplantation. This case was diagnosed cytologically as peritoneal PEL and confirmed histologically on peritoneal biopsies. The immune status for both HHV-8 and Epstein-Barr virus (EBV) was evaluated and showed positive immunostaining only for the former. The combination of the immunocytochemistry results with the existence of a clonal rearrangement in the immunoglobulin heavy chain gene (identified by PCR) was compatible with the diagnosis of PEL. The presence of T-cell markers was consistent with the diagnosis of PEL with an aberrant T-cell phenotype.
Primary effusion lymphoma (PEL) is a recently individualized form of non-Hodgkin's lymphoma (WHO classification) that mainly develops in HIV infected males, more frequently in homosexuals and advanced stages of the disease (total CD4+ lymphocyte count below 100-200/microL). Occasionally, it appears in other immunodepressive states (such as solid organs transplant period) and even, although very rarely, in immunocompetent patients. From a pathogenetic point of view, PEL has been related to Kaposi's sarcoma associated herpes virus (also named human herpesvirus 8, HHV-8), an etiological factor of Kaposi's sarcoma. The relative infrequency of this disease, the absence of wide casuistics allowing a better characterization, and its unfavorable outcome support the need of a deeper knowledge. We present here the clinical-biological findings of a patient, HIV seronegative, who was diagnosed with peritoneal PEL of T-cell origin, and not HHV-8-associated, five years after renal transplantation.
Background: Extramedullary hematopoiesis (EMH) is associated with a number of diseases in which the normal function of the bone marrow is disturbed. While organs with hemopoietic capacity like the liver and spleen are most commonly involved, EMH has also occasionally been found in other organs like the adrenal gland, lymph nodes, breast, thymus, small bowel and central nervous system. However, presentation of a myeloproliferative disorder, such as EMH in these organs is a rare event. Case Report: We report clinical and fine-needle aspiration (FNA) findings in a patient who presented with intrahepatic EMH which mimicked metastatic carcinoma from a colonic primary. Results: Ultrasound-guided FNA of the intrahepatic mass revealed megakaryocytes and myelocytes thus establishing the diagnosis of EMH. Conclusions: EMH is an unusual condition that can mimic other solid masses of the liver. Because radiologic findings are not specific, EMH should be considered in the differential diagnosis, especially in patients with a myeloproliferative disorder. FNA and subsequent cytopathological interpretation of the aspirates enables avoidance of unnecessary potentially hazardous surgery.
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