
ABSTRACT
Rationale and Objectives
Myelofibrosis is a rare progressive disease of the bone marrow characterized by the replacement of normal tissue with fibrous tissues. It can occur as a primary hematopoietic disorder or, more rarely, as a secondary condition.
Case
This is a case of a 51-year-old male who presented with right flank pain, generalized body weakness, and dizziness. Diagnostic work-up revealed bicytopenia (anemia and leukopenia), and a bone marrow biopsy confirmed the presence of myelofibrosis. Imaging studies and tissue biopsies revealed the presence of a renal mass consistent with clear cell renal carcinoma and rectosigmoid wall thickening consistent with a well-differentiated neuroendocrine tumor grade 2. Metastases were observed in the liver, lung, and adrenal glands. The patient's severe anemia required repeated transfusions, necessitating initial treatment with thalidomide and prednisone, leading to partial improvement of his cytopenias. Subsequently, systemic chemotherapy with Pazopanib was initiated; however, side effects of this treatment again led to anemia requiring red cell transfusion.
Discussion and Summary
Secondary myelofibrosis from metastatic nonhematopoietic malignancies is a rare progressive disease in which the bone marrow is replaced by fibrous tissues. Its diagnosis is based on clinical presentation, imaging investigation, and histopathology. Though the pathogenesis is still unknown, it is hypothesized to include hyperactivation of inflammatory signals and overproduction of inflammatory cytokines. The outcomes of patients with secondary myelofibrosis remain unclear due to its rarity, and the treatment of the primary disorder usually leads to resolution of marrow fibrosis. However, no data suggest that palliative chemotherapy may induce the resolution of myelofibrosis.