Mediastinal Adenopathy, Lung Infiltrates, and Hemophagocytosis
| Autor: | John K. Choi, Deborah W. Sevilla, Jerald Z. Gong |
|---|---|
| Rok vydání: | 2007 |
| Předmět: |
Lung Diseases
Pathology medicine.medical_specialty Anaplastic Lymphoma Mediastinal lymphadenopathy CD30 Activin Receptors Type II CD8 Antigens Ki-1 Antigen Lymphohistiocytosis Hemophagocytic Translocation Genetic Diagnosis Differential Fatal Outcome hemic and lymphatic diseases Humans Medicine Child Lymphatic Diseases Anaplastic large-cell lymphoma In Situ Hybridization Fluorescence Cytopenia business.industry Mediastinum Large-cell lymphoma General Medicine medicine.disease Immunohistochemistry Lymphoma Child Preschool Chromosomes Human Pair 2 Chromosomes Human Pair 5 Lymphoma Large-Cell Anaplastic Female Hemophagocytosis business |
| Zdroj: | American Journal of Clinical Pathology. 127:458-464 |
| ISSN: | 1943-7722 0002-9173 |
| DOI: | 10.1309/cqguuf5buvje0yu5 |
| Popis: | To date, only 1 report describes an anaplastic large cell lymphoma (ALCL) associated with hemophagocytosis in the pediatric population. To better characterize this unusual manifestation of ALCL, we identified 2 additional cases. Both patients had fever, cytopenia, decreased fibrinogen level, mediastinal or hilar adenopathy, minimal to no peripheral adenopathy, and lung infiltrates. Bone marrow biopsies and aspirates revealed striking hemophagocytosis but no ALCL. One patient fulfilled the criteria for hemophagocytic syndrome, but the other lacked 1 criterion. Both patients were initially given a misdiagnosis of infection-associated hemophagocytosis. Definitive diagnosis required lymph node biopsies that showed CD30+, anaplastic lymphoma kinase-I + ALCL. Both tumors responded to standard lymphoma chemotherapy. One patient achieved complete remission, whereas the other patient died of complications after 2 cycles of therapy. These findings are similar to the first reported case and indicate that pediatric ALCL can manifest with an unusual constellation of symptoms consisting of hemophagocytosis, mediastinal or hilar adenopathy, and lung infiltrates. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|