1. by Michele Paessler, and John Choi
Relapsed nodular sclerosis Hodgkin lymphoma and therapy-related myeloid sarcoma in a mediastinal mass
by Michele Paessler, and John Choi
Blood
Volume 123(9):
February 27, 2014
©2014 by American Society of Hematology

2. A 19-year-old man with nodular sclerosis Hodgkin lymphoma, diagnosed on mediastinal mass biopsy, was in remission after completing 12 weeks of Stanford V chemotherapy, which includes adriamycin, vincristine, nitrogen mustard, bleomycin, etoposide, and predn...
A 19-year-old man with nodular sclerosis Hodgkin lymphoma, diagnosed on mediastinal mass biopsy, was in remission after completing 12 weeks of Stanford V chemotherapy, which includes adriamycin, vincristine, nitrogen mustard, bleomycin, etoposide, and prednisone, followed by radiation to the mediastinum. Two years after diagnosis, the patient was found to have a mediastinal mass and surrounding enlarged lymph nodes. A biopsy demonstrated recurrent Hodgkin lymphoma (panels A-B) and a mononuclear cell infiltrate forming sheets and dissecting between collagen bundles, which immunophenotyped as myeloblasts (panels A-C). Mixed lineage leukemia (MLL) fluorescence in situ hybridization on the paraffin-embedded tissue from the mediastinal mass revealed an MLL rearrangement (panel D). A bone marrow aspirate and biopsy were performed and showed no morphologic evidence of acute myeloid leukemia (AML) or cytogenetic abnormalities, but reverse transcription-polymerase chain reaction for MLL breakpoints t(9;11) was positive in less than 1% of cells. This is an interesting case of nodular sclerosis Hodgkin lymphoma that relapsed after 2 years with concomitant myeloid sarcoma in the same biopsy. Therapy-related AML (TR-AML) with balanced translocations, as seen here t(9;11), is associated with topoisomerase II inhibitors (etoposide) and a short latency period and presents with AML without a preceding myelodysplastic phase, as was the case in this patient. Although TR-myelodysplastic syndrome/AML has been described in Hodgkin lymphoma, concomitant therapy-related myeloid sarcoma in the same site as recurrence has not been described. Although TR-myelodysplastic syndrome/AML has an overall poor prognosis, the prognosis of a myeloid sarcoma with minimal involvement of the marrow is unclear. At relapse, the patient was treated with 2 cycles of clofarabine, cyclophosphamide, etoposide, and dexamethasone and received a bone marrow transplant. (A) Hematoxylin and eosin (nodules separated by bands of fibrosis). In the nodules are classic Reed-Sternberg cells. The mononuclear cells forming sheets are the myeloid blasts. (B) CD30 highlights the Reed-Sternberg cells. (C) Myeloperoxidase highlights the myeloid blasts. (D) MLL split apart probe. The yellow signal is normal because they are fused; the red and green signals are separated because there is an MLL rearrangement. Panel D courtesy of Dr Susana Raimondi, St. Jude Children's Research Hospital.
Michele Paessler, and John Choi Blood 2014;123:1291
©2014 by American Society of Hematology