Chronic lymphocytic leukemia associated with bone marrow fibrosis: Possible role of interleukin 1α in the pathogenesis

Abstract

B-cell chronic lymphocytic leukemia (B-CLL) associated with bone marrow fibrosis is described. The conditioned medium of the CLL cells contained a high quantity of interleukin 1 alpha (IL-1 alpha), and showed growth promoting activity for marrow fibroblasts which was partially inhibited by anti IL-1 alpha antibody. In addition, the conditioned medium as well as IL-1 alpha stimulated the growth of marrow fibroblasts from the patient. These results strongly suggested the marrow fibrosis occurred by the secretion of IL-1 alpha from the CLL cells and its growth stimulation of marrow fibroblasts. As far as we know, this is the first case in which B-CLL was associated with marrow fibrosis, and its mechanism was investigated.

Essential Role of Matrix Metalloproteinases in Interleukin-1-induced Myofibroblastic Activation of Hepatic Stellate Cell in Collagen

“…However, it induces HSC proliferation (18) and expression of intercellular adhesion molecule-1 and chemokines in a nuclear factor-B-dependent manner (17,19). Although insufficiently documented for HSC activation, IL-1 is accepted as a potent cytokine for fibrosis of other organs such as the lung, (20) bone marrow (21), and kidney (22,23).…”

Coexistence of Chronic Lymphocytic Leukemia and Myeloproliferative Neoplasm

“…The simultaneous occurrence of lymphoproliferative and myeloproliferative disorder is very rare [1–4]. It is still under investigation whether these two clonal disorders are caused by one primary genetic alteration that increased genomic instability and susceptibility to further clonal disorders or are purely coincidental findings of a different origin [5–8]. …”

Simultaneous Presence of Two Hematological Malignancies: Chronic Lymphocytic Leukemia and Myelofibrosis in a Patient

“…Secondary myelofibrosis occurs in 20-30% of CLL cases, but usually does not manifest clinically [3]. Kimura et al have suggested that the marrow fibrosis occurs by the secretion of IL-1 alpha from the CLL cells causing growth stimulation of marrow fibroblasts [1]. A review of literature revealed anecdotal case reports of coexistent CLL with myelofibrosis [4].…”

“…Coexistence chronic lymphocytic leukemia (CLL) with myelofibrosis is a rare association, though CLL has been described to occur with solid tumors [1][2][3]. When these two coexist, either CLL may precede or follow idiopathic myelofibrosis [2,3].…”

“…Initial presentation of CLL with myelofibrosis is extremely unusual and remains a subject of anecdotal case reports [1][2][3]. Secondary myelofibrosis occurs in 20-30% of CLL cases, but usually does not manifest clinically [3].…”