Snapshot A 50-year-old man presents to his primary care physician for an annual visit. He has no complaints. His past medical history includes hypertension, hypercholesterolemia, and rheumatoid arthritis. He goes for routine blood tests today, which reveals marked pancytopenia. A peripheral blood smear is done, showing cells with hair-like projections. Introduction Indolent leukemia of mature B-cells with infiltration of bone marrow and spleen Epidemiology median onset 50-55 years of age male > female 4.5x Associated conditions autoimmune conditions Characteristics “hairy cells” with filamentous, hair-like projections tartrate-resistant acid phosphatase (TRAP) + Prognosis good prognosis, especially with good response to purine analogs Presentation Symptoms pancytopenia from infiltration of bone marrow fatigue infections bleeding anemia Physical exam splenomegaly from infiltration of spleen no lymphadenopathy Evaluation Peripheral blood smear B-cells with hairy projections + TRAP stain (tartrate-resistant acid phosphatase) Flow cytometry CD11c+ and CD2+ CD5- and CD10- Bone marrow aspirate dry tap due to marrow fibrosis CBC anemia thrombocytopenia leukopenia Differential Diagnosis Non-Hodgkin lymphoma Mantle cell lymphoma Treatment Purine analogs first line: cladribine (2-CDA) pentostatin both inhibit adenosine deaminase recall this is the same enzyme deficiency seen in SCID leading to B-cell dysfunction Complications Complications infections increased risk of malignancy
QUESTIONS 1 of 2 1 2 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (M1.ON.14.23) A 66-year old man presents to your clinic with a complaint of abdominal fullness and six months of worsening fatigue, weakness, and easy bruising. On physical exam, vital signs are stable, but you note marked splenomegaly and scattered ecchymoses. Labs are noteworthy for a hematocrit of 25%, total white blood cell count of 3000/uL, and platelet count of 35,000/uL. A peripheral blood smear is shown in Figure A; a tartrate-resistant acid phosphatase (TRAP) assay comes back positive. On further diagnostic workup, a bone marrow aspirate does not yield a sample due to extensive fibrosis. Which of the following is the first-line agent for the treatment of this condition? QID: 104663 FIGURES: A Type & Select Correct Answer 1 Vincristine 7% (19/256) 2 Rituximab 18% (47/256) 3 Cyclophosphamide 12% (30/256) 4 Cladribine 52% (132/256) 5 Methotrexate 9% (22/256) M 2 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic
All Videos (0) Oncology | Hairy Cell Leukemia (HCL) Oncology - Hairy Cell Leukemia (HCL) Listen Now 10:42 min 1/21/2023 0 plays 0.0 (0)