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![Syed Ali Abutalib, MD](/media/14013274/109-abutalib.jpg)
Syed Ali Abutalib, MD
![L. Jeffrey Medeiros, MD](/media/14013276/109-medeiros.jpg)
L. Jeffrey Medeiros, MD
The ASCO Post is pleased to present Hematology Expert Review, an ongoing feature that occasionally quizzes readers on issues in hematology. In this first-part of a two-part installment, Drs. Abutalib and Medeiros highlight the histologically rare lymphocyte-rich type of classic Hodgkin lymphoma, which accounts for about 5% of all classic Hodgkin lymphomas. See the second part that follows this one on mixed-cellularity classic Hodgkin lymphoma. For each quiz question that follows, select the one best answer. The correct answers and accompanying discussions follow.
Question 1
Which of the following statements about lymphocyte-rich classic Hodgkin lymphoma is correct?
A. This neoplasm has a female predominance.
B. This neoplasm usually presents with early-stage disease.
C. This neoplasm is usually observed in patients with HIV infection.
D. This neoplasm is the most common type of classic Hodgkin lymphoma.
Question 2
Which of the following statements about morphology in lymphocyte-rich classic Hodgkin lymphoma is correct?
A. This neoplasm is characterized by scattered Hodgkin/Reed-Sternberg cells in a cellular background of small lymphocytes and commonly has a nodular pattern.
B. This neoplasm is characterized by Hodgkin/Reed-Sternberg cells with lacunar-type morphology.
C. This neoplasm is characterized by scattered Hodgkin/Reed-Sternberg cells in a cellular background comprising neutrophils and eosinophils and commonly has a diffuse pattern.
D. All of the above
Question 3
Which of the following statements about immune histology in lymphocyte-rich classic Hodgkin lymphoma is correct?
A. CD30 is absent in 10% of cases.
B. Rosettes with a T follicular helper cell immunophenotype surrounding the neoplastic cells are present in all cases.
C. The background lymphocytes are mostly of T-cell lineage with a predominance of CD4-positive cells.
D. The background lymphocytes in the nodules have an immunophenotype similar to that of mantle zones.
Answers to Hematology Expert Review Questions
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Question 1
Which of the following statements about lymphocyte-rich classic Hodgkin lymphoma is correct?
Correct answer: B. This neoplasm usually presents with early-stage disease.
Expert Perspective
Classic Hodgkin lymphoma is a monoclonal lymphoid neoplasm derived from B cells and composed of mononuclear Hodgkin cells and multinucleated Reed-Sternberg cells in a background containing a variable mixture of non-neoplastic reactive immune cells, including small lymphocytes, eosinophils, histiocytes, and plasma cells.1,2 Approximately 90% of cases are classic Hodgkin lymphoma, and approximately 10% of cases are nodular lymphocyte-predominant Hodgkin lymphoma. There are four histologic types of classic Hodgkin lymphoma: nodular sclerosis, mixed cellularity, lymphocyte-rich, and lymphocyte-depleted. There is a male predominance for most classic Hodgkin lymphoma types (including lymphocyte-rich), with the exception being nodular sclerosis type, in which the incidence is approximately equal in men and women.
Mixed cellularity, not lymphocyte-rich, classic Hodgkin lymphoma is more frequent in patients with HIV infection and in developing nations. Nodular sclerosis accounts for most (approximately 70%) cases of classic Hodgkin lymphoma cases in Europe and in the United States.1,2 Lymphocyte-rich classic Hodgkin lymphoma accounts for approximately 5% of classic Hodgkin lymphomas, occurring at a frequency slightly less than that of nodular lymphocyte-predominant Hodgkin lymphoma. Compared with other histologic types, patients with lymphocyte-rich classic Hodgkin lymphoma are, on average, older and usually present with early stages of disease.3
Question 2
Which of the following statements about morphology in lymphocyte-rich classic Hodgkin lymphoma is correct?
Correct answer: A. This neoplasm is characterized by scattered Hodgkin/Reed-Sternberg cells in a cellular background of small lymphocytes and commonly has a nodular pattern.
Expert Perspective
Lymphocyte-rich classic Hodgkin lymphoma is characterized by scattered Hodgkin/Reed-Sternberg cells in a background of small lymphocytes that usually has a nodular pattern (Figure 1A) but uncommonly can have a diffuse pattern.2 The nodules can contain small reactive germinal centers, with the neoplastic Hodgkin/Reed-Sternberg cells in a mantle-zone like distribution (Figure 1B). The T zones of the lymph node are attenuated. Nodular sclerosis is characterized by collagen bands that surround at least one nodule and by Hodgkin/Reed-Sternberg cells with lacunar-type morphology.1 The background cells in nodular sclerosis include many granulocytes.
Lymphocyte-rich classic Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma are both nodular and have a background of small lymphocytes. However, the cells of the lymphocyte-rich type have a classic immunophenotype, and the neoplastic lymphocyte-predominant cells of nodular lymphocyte-predominant Hodgkin lymphoma are B cells positive for CD20 and CD45/LCA.
Question 3
Which of the following statements about immune histology in lymphocyte-rich classic Hodgkin lymphoma is correct?
Correct answer: D. The background lymphocytes in the nodules have an immunophenotype similar to mantle zones.
![](/media/14013277/110-table.jpg)
Expert Perspective
CD30 is expressed by the neoplastic cells in virtually all cases of classic Hodgkin lymphoma, including all cases of the lymphocyte-rich variant (Figure 1C). The neoplastic cells also commonly express CD15. Rosettes with a T follicular helper cell (TFH) immunophenotype (PD1/CD279+, CD57-/+) surrounding the neoplastic cells are present in as many as 50% of cases. The background lymphocytes in lymphocyte-rich classic Hodgkin lymphoma are mostly B cells positive for CD20, PAX-5, IgM, and IgD and are negative for CD10 and BCL-6, similar to mantle zone lymphocytes (See Table 1 for a brief overview of lymphocyte-rich classic Hodgkin lymphoma.)
DISCLOSURE: Dr. Abutalib is an advisor for AstraZeneca. Dr. Medeiros reported no conflicts of interest.
REFERENCES
1. Stein H, Pileri SA, Weiss LM, et al: Hodgkin lymphomas: Introduction, in Swerdlow SH, Campo E, Harris NL, et al (eds.): WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, pp 424-430. Lyon, France, International Agency for Research in Cancer, 2017.
2. Anagnostopoulos I, Piris MA, Isaacson PG, et al: Classic Hodgkin lymphoma, in Swerdlow SH, Campo E, Harris NL, et al (eds.): WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, pp 438-440. Lyon, France, International Agency for Research in Cancer, 2017.
3. Shimabukuro-Vornhagen A, Haverkamp H, Engert A, et al: Lymphocyte-rich classical Hodgkin’s lymphoma: Clinical presentation and treatment outcome in 100 patients treated within German Hodgkin’s Study Group trials. J Clin Oncol 23:5739-5745, 2005.