Anaplastic Large Cell Lymphoma, ALK Positive, Presenting With Leukemic Phase: A Case Report

• Published in: American journal of clinical pathology 2018-09, Vol.150 (suppl_1), p.S100-S100
• Main Authors: Carballo, Juan, Velez, Roman, Noy, Miguel, Arocho, Karina, Rivera-Rodriguez, Noridza
• Format: Article
• Language: English
• Subjects: Acute lymphoblastic leukemia; Anaplastic large-cell lymphoma; Biopsy; Blood flow; Bone marrow; Case reports; CD30 antigen; Cytology; Diagnosis; Flow cytometry; Leukemia; Leukocytosis; Lymph nodes; Lymphadenopathy; Lymphatic leukemia; Lymphocytes T; Lymphoma; Nuclei; Patients; Peripheral blood
• ISSN: 0002-9173; 1943-7722
• DOI: 10.1093/ajcp/aqy097.242

Abstract Purpose Anaplastic large cell lymphoma (ALCL) is a heterogeneous group of hematopoietic neoplasms composed predominantly of large cells with bizarre morphology, often with horseshoe-shaped nuclei and sinusoidal invasion. ALCL is a relatively rare lymphoma, accounting for 3% of all adult non-Hodgkin lymphomas. Leukemic presentation occurs in a minority of ALCL, and caution must be taken to avoid a diagnostic pitfall. Case Description We report the case of anaplastic large cell lymphoma, ALK positive, in a 47-year-old female patient who complained of fatigue, malaise, and shortness of breath of 1 week of evolution associated with persistent leukocytosis. Clinical Approach A peripheral blood flow cytometry was consistent with a T-cell acute lymphoblastic leukemia, and the patient was transferred to the San Juan University Hospital. Clinical Findings At our institution, the patient had prominent right cervical lymphadenopathy and multiple prominent periaortic, aortocaval, celiac axis, mesenteric, bilateral internal, and external iliac and bilateral inguinal region lymph nodes. Bone marrow aspiration and biopsy were consistent with 70% cellularity and diffuse infiltrate of small atypical cells with lobulated nuclei, positive for leukemia/lymphoma involvement. A right cervical lymph node was resected and showed a diffuse infiltrate of small lobulated cells with sinusoidal invasion. The cells were positive for ALK and CD30 immunostains, which was consistent with the diagnosis of anaplastic large cell lymphoma, ALK positive. Hypothesis Anaplastic large cell lymphoma with leukemic phase is a rare presentation and a diagnostic challenge for the pathologist. It mimics other entities that include adult T-cell leukemia lymphoma, T-cell lymphoblastic lymphoma, Sézary syndrome, and T-cell prolymphocytic leukemia, and a high index of suspicion is warranted for a correct diagnosis and proper treatment.