Case 14 - Discussion

Uploaded: 2007-06-27, Updated: 2008-01-01

CD57, 40×

The lymph node architecture is effaced by a nodular infiltrate composed predominantly of small lymphocytes with admixed histiocytes and L&H cells. The L&H cells are positive for EMA, CD45, CD20 and CD79a, and negative for CD15 and CD30. The nodules are composed by  B-cell lymphocytes (CD20 and CD79a positive) and CD3+ T-cells, many of them are CD57+ with  tendency to surround the L&H cells. CD23 is positive in FDCs. 

Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL)

CLINICAL FEATURES

• Monoclonal B-cell tumor, nodular or nodular/diffuse pattern, scattered large popcorn or LH cells;

• 5% of all HD cases;

• M>F, 30-50 YO.

MICROSCOPIC FINDINGS

• Lymph node is totally or partially replaced by a nodular or nodular and diffuse infiltrate;

• The nodules are predominantly consist of small lymphocytes, histocytes, and scattered LH cells;

• The LH cells are large with scant clear cytoplasm and one nucleus;

• The nuclei are often large, irregular, folded or multilobated or popcorn-like;

• The chromatin is mostly vesicular with a thin nuclear membrane;

• The nucleoli are often multiple, basophilic and smaller than those in classic RS cells (large eosinophilic);

• Many non-neoplastic CD57+ T cells may surround LH cells and form the rosette-like pattern;

• The architecture of the nodules is compose of a large spherical meshwork of follicular dendritic cells;

• Neutrophils and eosinophils are absent;

• One typical nodule is sufficient to exclude the diagnosis of T-cell rich large B-cell lymphoma.

DIFFERENTIAL DIAGNOSES

• T-cell rich large B-cell lymphoma: similar IPX profile, but no typical NLPHL nodules;

• DLBCL: less expression of EMA, no typical NLPHL nodules;

• ALCL: CD30+, CD2+, perforin/granzyme+, ALK+/-, CD43+/-, B-cell markers-.

• Reactive lymphoid hyperplasia: especially when tumor cells are scant. Need to spend some time in higher power.

IMMUNOHISTOCHEMISTRY AND SPECIAL STAINS

CYTOGENETIC STUDIES

• Ig clonal rearrangement;

• Tumor cells are EBV negative.

TREATMENT AND PROGNOSIS

• Stage I and II: 10-year overall survival > 80%.

REFERENCES

• WHO Pathology & Genetics. Tumours of Haematopoietic and Lymphoid Tissues. IARC Press, Lyon 2001