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Hodgkin's lymphoma classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2] Mohsen Basiri M.D.

Overview

Hodgkin’s lymphoma (HL) may be classified according to World Health Organization (WHO) classification into two major subgroups: nodular lymphocyte predominant and classic Hodgkin’s lymphoma. classic Hodgkin’s lymphoma is further divided into four subtypes: nodular sclerosis classic HL (NSHL), mixed cellularity classic HL (MCHL), lymphocyte rich classic HL (LRHL), and lymphocyte depleted classic HL (LDHL).

According to the Ann Arbor Staging System with Cotswolds modifications, there are 4 stages of Hodgkin’s Lymphoma based on clinical features gathered from history and physical examination, and findings on positron emission tomography/computed tomography (PET/CT) scan of the chest, abdomen, and pelvis. Each stage is assigned one letter and one number that designate the number of involved lymph node regions and the presence/absence of systematic symptoms or of bulky or extended disease .

Classification

Hodgkin’s lymphoma may be classified according to World Health Organization (WHO) classification into two major subgroups based upon the appearance and immunophenotype of the tumor cells:[1]

  • Nodular lymphocyte predominant Hodgkin’s lymphoma
  • Classical Hodgkin’s lymphoma

Classic Hodgkin’s lymphoma is further divided into four subtypes:

  1. Nodular sclerosis classic HL (NSHL)
  2. Mixed cellularity classic HL (MCHL)
  3. Lymphocyte rich classic HL (LRHL)
  4. Lymphocyte depleted classic HL (LDHL)
Classic Hodgkin’s lymphoma classification
Name Description
Nodular sclerosing Most common subtype and is composed of large tumor nodules showing scattered lacunar classical Reed–Sternberg cells set in a background of reactive lymphocytes, eosinophils, and plasma cells with varying degrees of collagen fibrosis/sclerosis.
Mixed-cellularity Common subtype and is composed of numerous classic Reed-Sternberg cells admixed with numerous inflammatory cells including lymphocytes, histiocytes, eosinophils, and plasma cells without sclerosis. This type is most often associated with Epstein–Barr virus (EBV) infection and may be confused with the early, so-called ‘cellular’ phase of nodular sclerosing classical Hodgkins lymphoma.
Lymphocyte rich Rare subtype, show many features which may cause diagnostic confusion with nodular lymphocyte predominant B-cell Non-Hodgkin’s Lymphoma (B-NHL). This form also has the most favorable prognosis.
Lymphocyte depleted Rare subtype, composed of large numbers of often pleomorphic Reed-Sternberg cells with only few reactive lymphocytes, which may easily be confused with diffuse large cell lymphoma. Many cases previously classified within this category would now be reclassified under anaplastic large cell lymphoma.
  • According to the Ann Arbor Staging System with Cotswolds modifications[2], there are 4 stages of Hodgkin’s Lymphoma based on clinical features gathered from history and physical examination, and findings on positron emission tomography/computed tomography (PET/CT) scan of the chest, abdomen, and pelvis. Each stage is assigned one letter and one number that designate the number of involved lymph node regions and the presence/absence of systematic symptoms or of bulky or extended disease . The stages of Hodgkin’s Lymphoma are shown in the table below:
Hodgkin’s Lymphoma Ann Arbor Staging with Cotswolds Modifications
Stage Definition
I Involvement of a single lymph node (I) or single extranodal site (IE)
II Involvement of two or more lymph node ereas on the same side of the diaphragm alone (II) or with involvement extralymphatic organ (IIE)
III Involvement of lymph node ereas or lymphoid structures on both sides of the diaphragm
IV Disseminated or multiple involvement of the extranodal organs

In the Ann Arbor Staging System with Cotswolds modifications, letters A and B indicate the absence (A) or presence (B) of one or more of the following systemic symptoms:

  • Fevers with temperatures above 38°C (>100.4°F) during the previous month

The subscript “X” in this staging system denotes bulky disease for treatment purposes.[3] Bulky mass is determined as a single mass of 10 cm or greater than one-third of the transthoracic diameter at any level of thoracic vertebrae as determined by computed tomography.[4]

References

  1. Swerdlow, S. H.; Campo, E.; Pileri, S. A.; Harris, N. L.; Stein, H.; Siebert, R.; Advani, R.; Ghielmini, M.; Salles, G. A.; Zelenetz, A. D.; Jaffe, E. S. (2016). “The 2016 revision of the World Health Organization classification of lymphoid neoplasms”. Blood. 127 (20): 2375–2390. doi:10.1182/blood-2016-01-643569. ISSN 0006-4971.
  2. Lister TA, Crowther D, Sutcliffe SB, Glatstein E, Canellos GP, Young RC; et al. (1989). “Report of a committee convened to discuss the evaluation and staging of patients with Hodgkin’s disease: Cotswolds meeting”. J Clin Oncol. 7 (11): 1630–6. PMID 2809679.
  3. Anita Kumar, Irene A. Burger, Zhigang Zhang, Esther N. Drill, Jocelyn C. Migliacci, Andrea Ng, Ann LaCasce, Darci Wall, Thomas E. Witzig, Kay Ristow, Joachim Yahalom, Craig H. Moskowitz & Andrew D. Zelenetz (2016). “Definition of bulky disease in early stage Hodgkin lymphoma in computed tomography era: prognostic significance of measurements in the coronal and transverse planes”. Haematologica. 101 (10): 1237–1243. doi:10.3324/haematol.2016.141846. PMID 27390360. Unknown parameter |month= ignored (help)
  4. Bruce D. Cheson, Richard I. Fisher, Sally F. Barrington, Franco Cavalli, Lawrence H. Schwartz, Emanuele Zucca & T. Andrew Lister (2014). “Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification”. Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 32 (27): 3059–3068. doi:10.1200/JCO.2013.54.8800. PMID 25113753. Unknown parameter |month= ignored (help)


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