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Lymphoma - Non B cell neoplasms

Hodgkin lymphoma

Lymphocyte-depleted classical Hodgkin lymphoma


Reviewers: Dragos Luca, M.D. and Lauren B. Smith, M.D. (see Reviewers page)
Revised: 7 February 2012, last major update August 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● LDCHL is a diffuse subtype of classic Hodgkin lymphoma (CHL) rich in Hodgkin Reed-Sternberg (HRS) cells or depleted in non-neoplastic lymphocytes (WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th Edition, Lyon 2008)

Terminology
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● 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
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● <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
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● 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
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● 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
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● 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 Classical Hodgkin lymphoma subtypes are treated similarly with either chemotherapy alone or chemotherapy plus external beam radiation

Micro description
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● 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

Micro images
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Various images


Diffuse fibrosis and a Reed-Sternberg cell


Numerous Hodgkin cells in a background of fibrosis


Reticular form (also called sarcomatous)


Diffuse fibrosis form


Reed-Sternberg cell staining

Other images: #1; #2; #3

Positive stains
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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)

Negative stains
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Reed-Sternberg cells: CD45, CD3

Differential diagnosis
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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
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Mod Path 2003;16:1141 (B cell marker expression), Oncologist 2009;14:739, Hematology Am Soc Hematol Educ Program 2006:266

End of Lymphoma - Non B cell neoplasms > Hodgkin lymphoma > Lymphocyte-depleted classical Hodgkin lymphoma


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