Primary myelofibrosis

A rare myeloproliferative neoplasm characterized by stem-cell derived clonal over proliferation of mature myeloid lineages, such as erythrocytes, leukocytes, and megakaryocytes, with variable degrees of megakaryocyte atypia, associated with reticulin and/or collagen bone marrow fibrosis, osteosclerosis, ineffective erythropoiesis, angiogenesis, extramedullary hematopoiesis, and abnormal cytokine expression.

Paraproteinemia

An abnormal immunoglobulin or part of an Ig (light chain) in the circulation. Paraproteins are typically produced by a clonal population of B-cell derived plasma cells.


Total: 2

                      


(per page)
PMID (PMCID)
3355902
MIXED_SAMPLE Middle Aged
Frequent association of idiopathic myelofibrosis with plasma cell dyscrasias.
Duhrsen U, Uppenkamp M, Meusers P, Konig E, Brittinger G.
Blut. 1988;56(3):97-102.
In the second patient the interval between diagnosis of idiopathic myelofibrosis and IgG-kappa paraproteinemia was 11 years.
3355902
MIXED_SAMPLE Middle Aged
Frequent association of idiopathic myelofibrosis with plasma cell dyscrasias.
Duhrsen U, Uppenkamp M, Meusers P, Konig E, Brittinger G.
Blut. 1988;56(3):97-102.
One patient with idiopathic myelofibrosis had primarily coexistent IgG-lambda paraproteinemia and increasing osteolytic lesions; histologic evidence of multiple myeloma, however, was insufficient.