Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL)
At a Glance
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a mature B-cell lymphoma. It is a type of Hodgkin lymphoma, but it is very different from classical Hodgkin lymphoma. It represents only 5% of Hodgkin lymphomas. It usually affects young adult males. It also usually involves lymph nodes and is more localized (lower stage disease) at diagnosis.
What Tests Should I Request to Confirm My Clinical Dx? In addition, what follow-up tests might be useful?
NLPHL is usually diagnosed by a lymph node biopsy. A core needle biopsy is often not sufficient, and an excisional surgical biopsy is required. This lymphoma has a nodular "lumpy bumpy" quality. Most of the cells in the lymph node are reactive small lymphocytes and histiocytes. The neoplastic cells are actually few in number. They are large B-cells, called LP cells (lymphocyte predominant cells), also known as L&H cells (lymphocytic and histiocytic Reed Sternberg variant cells) or popcorn cells.
Immunohistochemical staining of the biopsy is required for diagnosis. The LP cells are CD45+ and CD20+ in nearly all cases and EMA+ in about one-half of the cases. The LP cells are CD30- and CD15-. These features help to differentiate NLPHL from classical Hodgkin lymphoma. The LP cells are ringed by small reactive T-cells that are CD3+, CD4+, and CD57+. The large nodules have many reactive small CD20+ B-cells. Stains like CD21 can highlight the nodular expansion of dendritic cells. Staining for Epstein Barr virus (EBV) is usually negative.
Flow cytometry is usually nondiagnostic in NLPHL. Cytogenetic karyotype and fluorescence in situ hybridization (FISH) studies are not usually helpful. Molecular polymerase chain reaction (PCR) studies to look for IgH gene rearrangement do not usually detect the clonal B-cell population, unless the LP cells are individually selected by a special technique, such as microdissection (usually a research-based test and not routinely performed for this 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?
NLPHL can be very difficult to diagnose. Differentiating between NLPHL, classical Hodgkin lymphoma, and diffuse large B-cell lymphoma (T-cell/histiocyte-rich variant) can be very difficult. Many pathologists feel it is one of the most difficult lymphomas to correctly diagnose, and even experts sometimes disagree.
A process called progressive transformation of germinal centers (PTGC) has an association with NLPHL. It can be found before, during, or after a diagnosis is made. It is unclear, however, if PTGC is a precursor to NLPHL. Most patients who only have PTGC do not go on to develop Hodgkin lymphoma. NLPHL also has a relationship with diffuse large B-cell lymphoma, especially the T-cell/histiocyte-rich variant. Diffuse large B-cell lymphoma can be found in association with NLPHL at diagnosis, and a small percentage of patients with NLPHL progress on to diffuse large B-cell lymphoma. NLPHL and diffuse large B-cell lymphoma may have a clonal relationship, and current understanding includes the possibility that they may overlap.
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