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Case 69 - Discussion

Hematopathology Case

CD3

CD3

CD20

CD15

CD30

CD30

 

EBER-ISH

This is case of classic Hodgkin lymphoma (cHL), nodular sclerosis type, syncitial variant. Lymphoma cells typically form sheets, clusters or nodules pattern with central necrosis and abundant neutrophils. The tumor cells may appear to be cohesive, which can resemble carcinoma or sarcoma. The tumor cells in this case show weak expression of CD3, which may raise the suspicion for anaplastic large cell lymphoma (ALCL). However, the positivity for CD15 and EBER supports cHL. It would be better if additional immunostains could be performed, including CD43, MUM1 and PAX5. cHL mostly express MUM1 and PAX5, and is negative for CD43, whereas ALCL is on the opposite side.

Classic Hodgkin Lymphoma,

Nodular Sclerosis Type, Syncitial/Sarcomatous Variant

The Key Features

  • Most common subtype of HL (~70%); most common location, mediastinum (80%);

  • Nodular growth pattern with broad fibrous collagen bands, and lacunar cells;

  • CD30+, CD15+, BSAP+, CD45−, EMA−; EBV/LMP1+ in 10-40% cases.

CLINICAL FEATURES

  • Account for ~ 70% of Classic HL, median age 28 years, M=F;

  • Location: mediastinal 80%, spleen/lung 10%, bone marrow 3%;

  • Most patient present with Stage II disease, 40% with B-symptoms.

MICROSCOPIC FINDINGS

  • Nodular growth pattern with broad fibrous collagen bands surrounding at least one nodule;

  • The tumor contains a highly variable number of HRS cells, small lymphocytes and other reactive inflammatory cells;

  • HRS cells tend to have more lobated nuclei with smaller lobes and less prominent nucleoli. Lacunar cells due to retraction of the cytoplasm;

  • Eosinophils are often numerous.

SUBTYPES/VARIANT

  • Syncitial (sarcomatous, monomorphic) variant: tumor cells are in large aggregates or sheets with central necrosis containing abundant neutrophils. This variant may resemble metastatic carcinoma.

DIFFERENTIAL DIAGNOSES

  • Use of Immunophenotypic Studies in the Differential Diagnosis of HL

     

    ALK

    CD30

    EMA

    CD15

    CD3

    LCA

    BSAP

    TIA1

    Clusterin

    ALCL

    +

    +

    +

    ±

    +

    +

    +

    PC-ALCL

    +

    ±

    +

    ±

    HD

    +

    +

    +

    PTCL

    ±

    +

    +

    ±

    DLBCL

    ±

    ±

    +

    +

    ALCL, anaplastic large cell lymphoma; LCA, leukocyte common antigen (CD45); TIA-1, T-cell intracellular antigen; Hodgkin’s, Hodgkin’s lymphoma;

    PC-ALCL, primary cutaneous ALCL; PTCL, peripheral T-cell lymphoma, unspecified; DLBCL, diffuse large B-cell lymphoma.

IMMUNOHISTOCHEMISTRY AND SPECIAL STAINS

  • CD30+ in almost all cases, CD15+ in 75-85% cases;

  • EBV/LMP1+ in 10-40% cases, lowest incidence of all HL;

  • BSAP+ in ~90% cases (B-cell specific activator protein, product of PAX5 gene), usually weakly positive

  • CD20+ in 40% cases with variable intensity;

  • CD45−, EMA−, OCT2- and BOB1−.

CYTOGENETIC STUDIES

  • Monoclonal immunoglobulin gene rearrangement in >98% of cases, rarely T-cell receptor gene rearrangement.

TREATMENT AND PROGNOSIS

  • Slightly better than mixed cellularity or lymphocyte depleted subtype.

REFERENCES

  • Jaffe ES, Harris NL, Stein H, Vardiman JW, editors. Pathology and genetics of tumours of haematopoietic and lymphoid tissues. World Health Organization classification of tumours. Lyon (France): IARC Press; 2001.