Chronic neutrophilic leukemia (CNL) is a rare chronic myeloproliferative disorder of unknown etiology, characterized by sustained peripheral blood neutrophilia (>25 × 109 /L) and hepatosplenomegaly.[1,2] The bone marrow is hypercellular. No significant dysplasia is in any of the cell lineages, and bone marrow fibrosis is uncommon.[1,2] Cytogenetic studies are normal in nearly 90% of the patients. In the remaining patients, clonal karyotypic abnormalities may include +8,...
Historically, radiation therapy alone had been the primary treatment for patients with early favorable HL, often after confirmatory negative staging laparotomy. A randomized, prospective trial involving 542 patients with early favorable HL compared MOPP-ABV for three cycles plus involved-field radiation therapy (IF-XRT) with subtotal nodal radiation; with a median follow-up of 7.7 years, combined modality was favored in terms of 5-year event-free survival (98% vs. 74%, P < .001) and 10-year overall survival (97% vs. 92%, P = .001).[Level of evidence: 1iiA] The late mortality from solid tumors, especially in the lung, breast, gastrointestinal tract, and connective tissue, and from cardiovascular disease makes radiation therapy a less attractive option for the best-risk patients, who have the highest probability of cure and long-term survival.[2,3,4,5,6] Recent clinical trials have focused on regimens with chemotherapy and IF-XRT or with chemotherapy alone.
A randomized, prospective trial from the National Cancer Institute of Canada involving 123 patients with early favorable HL compared ABVD for four to six cycles to subtotal nodal radiation; with a median follow-up of 11.3 years, no difference was observed in event-free survival (89% vs. 86%; P = .64) or in overall survival (OS) (98% vs. 98%; P = 0.95).[Level of evidence: 1iiA]
In a randomized study from the Milan Cancer Institute of patients with clinical early-stage HL, 4 months of ABVD followed by either IF-XRT or extended-field radiation therapy (EF-XRT) showed similar OS and freedom-from-progression with a 10-year median follow-up, but the study had inadequate statistical power to determine noninferiority of IF-XRT versus EF-XRT.[Level of evidence: 1iiDii]
The German Hodgkin Lymphoma Study Group (GHSG) randomly assigned 1,190 patients with early favorable HL to the following: