Lymphoma and plasma cell neoplasms
Hodgkin lymphoma
Lymphocyte depleted classic Hodgkin lymphoma
Copyright: 2002-2020, PathologyOutlines.com, Inc.
PubMed Search: "Lymphocyte depleted" classic Hodgkin lymphoma
Hodgkin lymphoma
Lymphocyte depleted classic Hodgkin lymphoma
Authors: Dragos C. Luca, M.D., Lauren Smith, M.D.
Topic Completed: 1 August 2011
Minor changes: 2 April 2020
Copyright: 2002-2020, PathologyOutlines.com, Inc.
PubMed Search: "Lymphocyte depleted" classic Hodgkin lymphoma
Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Clinical features | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Differential diagnosis | Additional referencesCite this page: Luca D, Smith L. Lymphocyte depleted classic Hodgkin lymphoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomanonBlymphocytedepleted.html. Accessed May 27th, 2020.
Definition / general
- LDCHL is a diffuse subtype of classic Hodgkin lymphoma (CHL) rich in Hodgkin Reed-Sternberg (HRS) cells or depleted in nonneoplastic lymphocytes (WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th Edition, Lyon 2008)
Terminology
- Most historic cases are actually anaplastic non-Hodgkin lymphoma, diffuse large B cell lymphoma or syncytial variant of nodular sclerosing Hodgkin lymphoma (Leuk Lymphoma 2009;50:937)
Epidemiology
- < 1% of all classic Hodgkin lymphoma in Western countries (rarest subtype); more common in developing countries
- Often HIV associated
- Median age 30 - 37 years
- Male predominance (60 - 75%)
Sites
- 90% have subdiaphragmatic disease or organomegaly
- Predilection for retroperitoneal lymph nodes, abdominal organs and bone marrow
- Marrow involvement is common (54%); 50% have peripheral adenopathy
Clinical features
- More advanced stage (III - IV) and with B symptoms at presentation
- More aggressive than other subtypes, including in HIV+ patients (Am J Clin Pathol 2004;121:727)
- Patients often present with B symptoms (N Engl J Med 1973;288:751)
- May be part of a continuum with mixed cellularity subtype (Clin Lymphoma Myeloma 2009;9:206, Cancer J 2009;15:129)
Treatment
- Aggressive course prior to modern therapy, still seen in parts of Europe and developing countries
- Comparable with other classic Hodgkin lymphoma in Western countries
- Poor prognosis in HIV cases
- All Classic Hodgkin lymphoma subtypes are treated similarly with either chemotherapy alone or chemotherapy plus external beam radiation
Microscopic (histologic) description
- Either diffuse fibrosis or reticular forms
Diffuse fibrosis form
- Complete effacement of nodal architecture by abundant disorderly connective tissue with PAS+ fibrinoid material and hypocellular background
- Rare Reed-Sternberg cells
- Classify as nodular sclerosis classic Hodgkin lymphoma if nodular and sclerotic
Reticular form
- No disorderly connective tissue, numerous bizarre Reed-Sternberg cells, often in sheets, with few lymphocytes
Bone marrow
- Rare Reed-Sternberg cells in amorphous, nonbirefringent eosinophilic background material and inflammatory infiltrate
- Multiple sections / levels often required for diagnosis
- Uninvolved marrow is normocellular with increased eosinophils (Am J Surg Pathol 1986;10:219)
- May resemble mixed cellularity classic Hodgkin lymphoma
- Sarcomatous pattern with pleomorphic HRS cells difficult to differentiate from anaplastic large cell lymphoma
- May have coagulative necrosis or sinusoidal invasion
Microscopic (histologic) images
AFIP Images
Diffuse fibrosis and a Reed-Sternberg cell
Numerous Hodgkin cells in a background of fibrosis
Images hosted on other servers:
Various images
Reticular form (also called sarcomatous)
Diffuse fibrosis form
Reed-Sternberg cell staining
Other images
Positive stains
- Reed-Sternberg cells: CD15, CD30, Fascin, variable CD20
- Most HIV positive cases are EBV+ (Am J Pathol 1993;142:1073, Virchows Arch 2008;453:611)
Differential diagnosis
- Anaplastic large cell lymphoma: anaplastic cells may resemble Reed-Sternberg cells but are CD2+, CD3+, CD45+; have t(2;5); are CD30- and PAX5-
- Diffuse large B cell lymphoma: no Reed-Sternberg cells; usually not CD15+; T cell / histiocyte rich variant may be CD30+
- Nodular sclerosis classic Hodgkin lymphoma syncytial type: well defined birefringent fibrous bands of acellular collagen plus cohesive aggregates of atypical mononuclear Reed-Sternberg cells
Additional references
- Mod Path 2003;16:1141 (B cell marker expression), Oncologist 2009;14:739, Hematology Am Soc Hematol Educ Program 2006:266
