ABSTRACT
Background: Plasma cell leukemia (PCL) is a rare, yet aggressive plasma cell (PC) neoplasm, variant of multiple myeloma (MM), characterized by high levels of PCs circulating in the peripheral blood. PCL can either originate de novo (primary PCL) or as a secondary leukemic transformation of MM (secondary PCL) and is characterized by circulating PCs >2×109/L in peripheral blood and a peripheral blood plasmacytosis >20%. Aims & Objective: Present study was undertaken to analyze the main clinical & pathological features of PCL. For diagnostic purpose the morphological appearances and confirmation by immunophenotyping are emphasized rather than more sophisticated testing methods that may not be widely available. Material and Methods: A descriptive study was carried out in the department of Pathology, in a tertiary care teaching hospital, Ahmedabad, India during year 2009-2013. We investigated the important clinical characteristics, pathological, biochemical & radiological features, immunophenotype, & prognostic factors of 7 patients of PCL. Results: Common clinical features at diagnosis were anaemia, renal insufficiency, bone pain, splenomegaly or hepatomegaly. Anaemia, leucocytosis, thrombocytopenia & plasmacytosis were seen in peripheral blood. Plasma cell marker - CD 38 & CD 138 were expressed in all cases. Serum β2-microglobulin, serum LDH were increased & serum albumin was decreased in all 7 cases & were associated with poor prognosis. The median survival time from diagnosis was 9 months. Conclusion: Plasma cells have characteristic morphological features which can be easily identified on peripheral blood & bone marrow examination.CD 38 & CD 138 are excellent plasma cell markers. Increased serum β2-microglobulin & serum LDH & decreased serum albumin are potent poor prognosis factors. PCL is aggressive neoplasm with poor response to chemotherapy & low median survival time from diagnosis.
ABSTRACT
La Histiocitosis de Células de Langerhans es un grupo de enfermedades con un compromiso mono o multisistémico; presenta manifestaciones clínicas y complicaciones que varían según el tejido afectado y la extensi¢n de la lesión; caracterizada por la proliferación de células de Langerhans. Se reporta el caso de una paciente femenina de 2 años y medio de edad, que presenta una masa de 6-7 cm en la zona parietal izquierda, dolorosa, que deforma la anatomía craneal; el cuadro evolucionó en 22 días y asoció anorexia. Un estudio por tomografía axial computarizada, mostró una fractura del hueso parietal asociada a un tumor homogéneo sólido en los tejidos blandos adyacentes, sin calcificaciones. Se realizó una resecci¢n tumoral, craneotomía y craneoplastía. Los estudios inmunohistoquímicos demostaron células S100, CD1a y CD68 positivas...
Subject(s)
Humans , Female , Infant , Histiocytosis, Langerhans-Cell/diagnosis , Histiocytosis, Langerhans-Cell/physiopathology , Histiocytosis, Langerhans-Cell/pathology , Costa RicaABSTRACT
Se ha efectuado una revisión de los trabajos sobre la fisiopatología del MM y sobre el tratamiento de las lesiones osteoporóticas que se presentan en casi todos los casos. Es sabido que los bifosfonatos son sumamente efectivos por lo que se comentan los actuales esquemas de tratamiento tomando en cuenta los recientes consensos. Hay un acuerdo prácticamente unánime en la eficacia del pamidronato y el zoledronato endovenosos aplicados mensualmente durante dos años, siendo aceptado el uso del clodronato oral en Europa pero no en EE.UU. Aunque mejorarían la calidad de la sobrevida, los bifosfonatos no la prolongarían. Se refieren las precauciones que habría que tomar para evitar la osteonecrosis de mandíbula, una complicación de las dosis altas de bifosfonatos que se ha comunicado con mayor frecuencia durante los últimos años. Se destaca la importancia de la consulta odontológica frecuente y del permanente cuidado dental.
The aim of this review is to discuss recent findings in the physiopathology and treatment of osteoporotic lesions present in almost all patients with MM. The efficacy of bisphosphonates is well known, so we summarize the current treatment schedules according to the most recent consensus. Pamidronate and Zoledronate are equally effective and universally accepted. They should be administered intravenously on a monthly basis for two years. Oral clodronate is accepted in Europe but not in USA. Even if bisphosphonates provide a better quality of life, they do not increase survival. Because osteonecrosis of the jaw has been repeatedly reported after high doses of bisphosphonates, we discuss the necessary precautions to prevent this condition emphasizing frequent dental care and examinations.
ABSTRACT
Histiocytosis is a term applied to a group of rare disorders of the reticuloendothelial system. Langerhan cell histiocytosis (LCH) is characterized by the clonal proliferation of a special type of cell, the Langerhans cell. These are dendritic antigen-presenting cells that are normally distributed in many organs. LCH is presents as one of three clinicopathologic entities: acute disseminated Langerhan cell histiocytosis (Letterer- Siwe syndrome), unifocal eosinophilic granuloma, or multifocal eosinophilic granuloma. Unifocal lesions usually affect the skeletal system and may be asymptomatic, may cause pain and tenderness and in some instances, pathological fractures. This disorder is relatively indolent and may regress spontaneously or needs local excision or irradiation. We present a case of solitary eosinophilic granuloma of frontal bone.