Pathological rupture of the spleen in uncomplicated multiple myeloma is an extremely rare phenomenon, which has been reported only in 2 cases. In our patient, the massive liver and spleen infiltration led to a situation in which bleeding diathesis and mechanical stress can be assumed as the causative factors of the spleen rupture. Thus, the marked propensity to extramedullary infiltration of myeloma cells in this case seems to be responsible for the unfortunate outcome of the patient. The results of the immunophenotypical and genetic analysis of these myeloma samples suggest that events affecting cell relationships with the microenvironment might be related to the ability of neoplastic cells to grow outside bone marrow and to infiltrate extramedullary organs. Multiple myeloma is usually considered a slowly progressing disease with a long natural history. The case we reported, with its sudden and fatal outcome, highlights the importance of the clinical evaluation of extramedullary localization of the disease. Further studies are needed to evaluate the role of molecular alterations of cell cycle progression and microenvironment interactions with the aim of identifying possible therapeutic targets.
Fatal Splenic Rupture in a Previously Undiagnosed Multiple Myeloma: Morphological, Immunophenotypical and Molecular Cytogenetic Analyses
CAMPIOTTI, LEONARDO;SESSA, FAUSTO;UCCELLA, SILVIA
Ultimo
2013-01-01
Abstract
Pathological rupture of the spleen in uncomplicated multiple myeloma is an extremely rare phenomenon, which has been reported only in 2 cases. In our patient, the massive liver and spleen infiltration led to a situation in which bleeding diathesis and mechanical stress can be assumed as the causative factors of the spleen rupture. Thus, the marked propensity to extramedullary infiltration of myeloma cells in this case seems to be responsible for the unfortunate outcome of the patient. The results of the immunophenotypical and genetic analysis of these myeloma samples suggest that events affecting cell relationships with the microenvironment might be related to the ability of neoplastic cells to grow outside bone marrow and to infiltrate extramedullary organs. Multiple myeloma is usually considered a slowly progressing disease with a long natural history. The case we reported, with its sudden and fatal outcome, highlights the importance of the clinical evaluation of extramedullary localization of the disease. Further studies are needed to evaluate the role of molecular alterations of cell cycle progression and microenvironment interactions with the aim of identifying possible therapeutic targets.
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