A case of chronic lymphocytic leukemia overwhelmed by rapidly progressive idiopathic myelofibrosis

Abstract

Chronic lymphocytic leukemia (CLL) is sometimes associated with solid tumors but rarely coexists with other hematologic neoplasias in the same patient. There are isolated case reports in the literature of an association between CLL and idiopathic myelofibrosis, a representative disease of the group of the myeloproliferative syndromes. We describe the case of a 70-years old female diagnosed as having CLL and idiopathic myelofibrosis in a prefibrotic phase with an indolent course, managed only with observation. Twenty-eight months after diagnosis, the patient developed hepato-splenomegaly and progressivly rising of serum lactic dehydrogenase (LDH) levels; immature granulocytic cells and tear drop red cells appeared in the blood. A bone marrow trephine biopsy (after a "dry tap" external aspiration) was consistent with the diagnosis of overt idiopathic myelofibrosis and only residual foci of CLL cells were present. Three months later, the blood diagnostic features of CLL remained but a progressive fall in the numbers of CD5+/CD19+ cells was noted. Other observations related to this association in several chronological sequences, their possible pathogenesis possibilities, and the diagnostic value of the rise in serum LDH levels, are discussed. The case reported here constitutes an extremely rare situation of CLL overwhelmed by rapidly progressing idiopathic myelofibrosis.