Case 38 - Discussion

Uploaded: 2008-01-09, Updated: 2008-01-09

 

 

LCA CD5
CD20 CD20
CD79a CD79a
CD15 CD30
   
  • Case Description: The lymph node structure show diffuse infiltration of lymphoid cells. There are many scattered large cells within a mixed background of small lymphocytes and histiocytes. The large cells reveal large irregular nucleus with prominent nucleoli and vesicular chromatin. Multiple nucleation is common. The large neoplastic cells are positive for CD45, CD20, and CD79a but negative for EBV-LMP1, CD15 and CD30. The vast majority (>90%) of the small lymphocytes are positive for T cell markers (CD3, CD5) and the rest of them are positive for B cell markers (CD20 and CD79a). By kappa/lambda in situ hybridization, the ratio of kappa and lambda in large cells is >10:1.
  • Flow cytometry did not detect an abnormal B-cell population. In addition, molecular studies did not detect a clonal immunoglobulin heavy chain gene rearrangement.
  • Diagnosis: LYMPH NODES WITH HODGKIN'S DISEASE, NODULAR LYMPHOCYTE PREDOMINANT TYPE, WITH FOCAL DIFFUSE AREAS.
   
Nodular Lymphocyte Predominant Hodgkin Lymphoma

The Key Features

  • Nodular infiltrate with scattered neoplastic LH cells (CD19+, CD79a+, BCL6+, EMA+, OCT2+, CD15-, CD30-, EBV-);

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

  • CD57+ T cells surround LH cells and form the rosette-like pattern;

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

CD30

CD15

CD45

CD20

CD79a

BSAP

EMA

BCL6

ALK

CD43

J Chain

Ig

CD2

CD3

OCT2

BOB1

Perforin

Granzyme B

+

+

+

+

+/-

+

+/−

+/−

+bright

+/−

+

+

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