Case Report: A 61-year-old African American man presented to his PCP complaining of severe weakness and dyspnea with minimal exertion. The symptoms had begun about 6 weeks prior to the visit and had slowly worsened. The patient denied any bleeding, fever chills or sweats. He had a past, presumed history of ulcerative colitis, inactive, on no medications, as well as benign prostatic hypertrophy (BPH) without therapy. He denied any use of OTC’s; no drug or alcohol use. No risk factors for HIV. In the office he was noticed to be severely anemic with a Hgb of 2.5, on repeat was 2.7 gm/dL. Platelet count was 10 000/uL, confirmed on repeat CBC and examination of the smear. WBC was 4,500 with about 80% normal appearing lymphocytes. LDH was 143(wnl). A B12 level was low at 208, with an elevated MMA of 829. The patient was admitted for evaluation and transfusions. There were no petechiae or echymoses in the visible areas of the skin. The patient initially refused physical exam or any diagnostic procedures. A subsequent flow cytometric assay of his blood was consistent with CLL. Eventually a bone marrow aspirate and biopsy were performed. It showed a hypercellular bone marrow, with major decrease in all myeloid, erythroid and megakaryocytic elements which were replaced by population of mature, small lymphocytes, consistent with the diagnosis of CLL. In particular, histology was characterized by almost complete absence of megakaryocytes, leading to suspicion of an auto-immune component driving disease. The patient was started on pulse dexamethasone vitamin B12, and administered weekly anti-CD20 (Rituximab) for 4 doses. He was also started on eltrombopag and a BTK inhibitor (Ibrutinib) both of which he continues to take to date. His counts have slowly risen, and the patient continues to improve.
Discussion: Severe pancytopenia as a presentation of CLL is uncommon, perhaps in contrast to autoimmune cytopenias which are relatively common events during the lifecycle of patients with CLL and are thought to arise from antibody production by the normal BCells, in this setting of T-cell dysregulation. Given recent trends in the treatment of CLL with BTK inhibitors, which have been both used to treat, as well as believed to have caused auto-immune CLL complications it’s important to review the occurrence of cytopenias and how to manage them in this setting.
