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The pathologic and clinical heterogeneity of lymphocyte-depleted Hodgkin's disease
Insights
Lymphocyte-depleted Hodgkin's disease (LDHD) prognosis is not poor when accurately diagnosed. Misdiagnosis of aggressive non-Hodgkin's lymphomas (NHL) as LDHD may explain earlier reports of poor outcomes for this Hodgkin's disease subtype.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Lymphocyte-depleted Hodgkin's disease (LDHD) has been associated with a poor prognosis.
- Histologic diagnosis of LDHD can be challenging, often confused with aggressive non-Hodgkin's lymphomas (NHL).
- This diagnostic difficulty may contribute to earlier reports of LDHD's aggressive nature.
Purpose of the Study:
- To re-evaluate the prognosis of LDHD.
- To clarify the distinction between LDHD and NHL.
- To assess the impact of accurate diagnosis on treatment outcomes.
Main Methods:
- Retrospective review of 43 patients originally classified as LDHD treated with MOPP chemotherapy at the National Cancer Institute (NCI) between 1964 and 1976.
- Re-evaluation of initial diagnostic biopsies by pathologists without knowledge of clinical features.
- Subclassification of Hodgkin's disease subtypes and NHL.
Main Results:
- Of 39 reviewed biopsies, 10 were reclassified as NHL, 9 as LDHD, and 13 as nodular sclerosing Hodgkin's disease of the lymphocyte-depleted subtype (NSLD).
- NHL patients had a poor response to MOPP, with only 3 complete remissions (CR) and a median survival of 7 months.
- LDHD and NSLD groups achieved high CR rates (67% and 85%, respectively) with no reached median survival after 14 years of follow-up.
Conclusions:
- Accurately diagnosed and treated LDHD has a prognosis comparable to other Hodgkin's disease subtypes.
- Misclassification of aggressive NHL as LDHD likely accounts for historical reports of poor LDHD outcomes.
- Caution is advised in diagnosing LDHD, especially in patients with atypical clinical presentations.
Abstract:
Patients with Hodgkin's disease of the lymphocyte-depleted subtype (LDHD) have been said to have a poor prognosis. However, reports of this subtype are complicated by the fact that the histologic diagnosis of LDHD is often not straightforward, and its distinction from aggressive non-Hodgkin's lymphomas (NHL) can be difficult. We have reviewed our patients with LDHD at the National Cancer Institute (NCI) in light of an additional decade of experience with neoplastic and non-neoplastic conditions mimicking Hodgkin's disease. Of 198 patients who received MOPP (mechlorethamine, vincristine, procarbazine, prednisone) treatment at the NCI for Hodgkin's disease between 1964 and 1976, 43 (22%) were originally classified as LDHD. The initial diagnostic biopsies from 39 of these patients were reviewed and revealed ten with NHL, nine with LDHD, and 13 with nodular sclerosing Hodgkin's disease of the lymphocyte-depleted subtype (NSLD). The other seven patients had Hodgkin's disease without a lymphocyte-depleted component. The NHL patients were further subclassified as diffuse, large-cell (two cases) and large-cell, immunoblastic (eight cases). The pathologic review was done without knowledge of clinical features which were examined after review in the three major subgroups. Of ten patients with NHL, only three had a complete remission (CR), and median survival was 7 months. Nine of the NHL patients presented with features that are unusual for patients with Hodgkin's disease, such as bulky abdominal disease, epitrochlear lymphade-nopathy, or hypercalcemia. CRs were attained by 67% and 85% of patients in the LDHD and NSLD groups, respectively: median survival had not been reached in either group with a median of 14 years of follow-up. Lymphocyte-depleted Hodgkin's disease, adequately treated, is in our limited group of patients no worse than other histopathologic subtypes of Hodgkin's disease. The erroneous inclusion of patients with high-grade NHLs into this subtype of Hodgkin's disease may be one reason for earlier literature reports of its more aggressive nature. The diagnosis of LDHD should be made cautiously, particularly in patients with clinical features that are unusual for Hodgkin's disease at presentation.