Lymphocyte-Depleted Classical Hodgkin Lymphoma (LDCHL)

At a Glance

Lymphocyte-depleted classical Hodgkin lymphoma (LDCHL) is the rarest subtype of classical Hodgkin lymphoma (CHL), comprising less than 1% of cases in North America and Europe. It occurs more frequently in HIV infected individuals and in developing countries. LDCHL patients present more often with disseminated disease than patients with other subtypes of CHL, with frequent involvement of bone marrow (up to 75% of cases) and consequent cytopenias. Most patients exhibit systemic "B" symptoms (i.e., fever, night sweats, and/or significant weight loss).

Some patients, particularly the elderly or immunocompromised, may present with cytopenias, and the diagnosis may be made initially on bone marrow biopsy; such patients are usually subsequently found to also have nodal disease (particularly retroperitoneal) on staging studies. A cytopenic presentation with bone marrow as the first site of biopsied disease occurs in 10-15% of HIV infected patients diagnosed with CHL, and such cases are often of the LDCHL subtype.

What Tests Should I Request to Confirm My Clinical Dx? In addition, what follow-up tests might be useful?

A biopsy of involved tissue should confirm a clinical suspicion of LDCHL.

After diagnosis, the disease should be staged; advanced stage disease is common in LDCHL (see chapter on Classical Hodgkin lymphoma).(Table 1)

Table 1

Test Results Indicative of the Disorder
Tissue Biopsy Immunohistochemistry on Tissue Biopsy
Presence of numerous Reed-Sternberg cells admixed with sparse inflammatory cells. Reed-Sternberg cells are CD30+, CD45-, often CD15+, and PAX5+, but are usually negative for other B-cell markers.
Alternatively, less frequent Reed-Sternberg cells may be present within a fibroblastic background.

Are There Any Factors That Might Affect the Lab Results? In particular, does your patient take any medications - OTC drugs or Herbals - that might affect the lab results?

As with all types of CHL, a fine needle aspiration, limited tissue biopsy, such as a core biopsy, or biopsy of a small, partially involved node may yield false-negative results. This is even more of a problem with LDCHL in which the numerous Reed-Sternberg cells and mononuclear variants may suggest a diagnosis of a large cell non-Hodgkin's lymphoma or the numerous fibroblasts in some cases may lead to misdiagnosis of a soft tissue sarcoma.

What Lab Results Are Absolutely Confirmatory?

The Reed-Sternberg cells and mononuclear variants resemble those in other CHL subtypes but usually occur more frequently in a background that contains few non-neoplastic inflammatory cells. The immunophenotype of the Reed-Sternberg cells is identical to that of other CHL subtypes. In some cases of LDCHL, the Reed-Sternberg cells are infrequent, but the background is sparse in inflammatory cells and consists mostly of fibroblasts. Unlike the fibrosis of nodular sclerosis CHL, the fibrosis in LDCHL is more cellular and there are no collagenous fibrous bands.

What Tests Should I Request to Confirm My Clinical Dx? In addition, what follow-up tests might be useful?

Many cases exhibit Epstein-Barr virus (EBV) within the neoplastic Reed-Sternberg cells, as detected by either EBV-encoded RNA (EBER) in situ hybridization or by EBV latent membrane protein-1 (LMP1) immunohistochemistry.

Because of the frequency of the neoplastic Reed-Sternberg cells in most cases of LDCHL, an IGH gene rearrangement may be detected by polymerase chain reaction (PCR) studies; this result should not lead to misclassification as diffuse large B-cell lymphoma.

Are There Any Factors That Might Affect the Lab Results? In particular, does your patient take any medications - OTC drugs or Herbals - that might affect the lab results?

In small biopsies, the sheets of Reed-Sternberg cells may mimic a non-Hodgkin's lymphoma. In particular, the tumor can resemble anaplastic large cell lymphoma (ALCL), a type of T-cell lymphoma that shares CD30 expression with CHL. However, unlike most cases of ALCL, the neoplastic cells in LDCHL lack expression of any T-cell markers and should express PAX5. In addition, CD15 and stains for EBV, which should be negative in ALCL, are often positive in LDCHL. Anaplastic lymphoma kinase (ALK) immunohistochemistry is uniformly negative in LDCHL but is positive in many cases of ALCL.

In cases presenting in the bone marrow, particularly in HIV infected patients, prominent background fibrosis can render the Reed-Sternberg cells difficult to identify if the diagnosis of CHL is not being considered. Thus, LDCHL should be considered in the differential diagnosis of unexplained diffuse or focal marrow fibrosis in cytopenic patients, particularly in immunosuppressed individuals.

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