NCI Working Formulation for Lymphomas

• Low-grade

  • 50 – 70% 5YS

  • Tend to be widespread at diagnosis, indolent, and poorly responsive to chemo (due to slow growth)

  • Mostly in older folks

  • May transform to a higher grade lymphoma called Richter’s syndrome

  • If the spleen is involved, only the white pulp is affected; if the liver is involved, only the periportal zone is affected (both of these areas are where lymphocytes are seen in these organs)

  • All are associated with t(14; 18), which involves bcl-2 overexpression

  • If > 75% of the growth pattern is follicular, there is a longer survival regardless of cell type; all follicular patterns can become diffuse over time (shortens survival time)

  • Malignant lymphoma, small lymphocytic

    • This lymph node counterpart to CLL is the only diffuse low-grade lymphoma

    • Preferentially involves nodes, marrow, and peripheral blood

    • The more big cells the see, the worse the prognosis

  • Malignant lymphoma, follicular, mostly small cleaved cells

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    • Follicular growth pattern

  • Malignant lymphoma, follicular, mixed small cleaved and large cell – mixture of small cleaved and large cleaved and/or large non-cleaved cells

• Intermediate-grade

  • 35-45% 5YS

  • May transform to higher grade

  • Malignant lymphoma, follicular, predominately large cell

    • > 50% large cleaved or noncleaved cells

    • Only follicular intermediate grade lymphoma

  • Malignant lymphoma, diffuse, small cleaved cell

  • Malignant lymphoma, diffuse, mixed small and large cell

    • Cells of both sizes may be cleaved and/or non-cleaved

    • Mostly B cells

  • Malignant lymphoma, diffuse, large cell cleaved and/or non-cleaved – most common intermediate grade

• High-grade

  • 23-32% 5YS

  • Tend to be localized at diagnosis, aggressive, but up to 60% respond to chemo

  • Liver or spleen involvement characterized by large destructive masses

  • Malignant lymphoma, large cell immunoblastic

    • Look like plasma cells (“plasmacytoid”), with discrete cytoplasmic margins, eccentric nucleus, and prominent nucleolus

    • Localized or extranodal disease more common; marrow, liver, and spleen not to often involved

    • Mostly B cells

    • About 50% are associated with a history of autoimmune disease or immunosuppression

  • Malignant lymphoma, lymphoblastic

    • Most common lymphoma in kids

    • Frequently associated with mediastinal mass, early marrow involvement, and progression to ALL

    • Very aggressive

    • Mostly T cells

  • Malignant lymphoma, small non-cleaved (Burkitt’s)

    • Intermediate in size between small mature lymphocytes and large non-cleaved cells

    • Starry-sky pattern with frequent mitoses

    • Burkitt’s type is associated with EBV exposure

• Miscellaneous

  • Mantle zone (intermediate differentiated) lymphoma

    • This is a high-grade malignant lymphoma

    • Morphology, however, looks like low-grade, so need to pick this one up

    • Arise from memory or virgin B cells in the mantle zone around the follicular center (which is crushed when the cells begin expanding)

  • Hairy cell leukemia

    • Partial monocytic derivation

    • Seen in the red pulp of the spleen because that’s where the monocytes live (also in marrow and peripheral blood)

  • Monocytoid B cell lymphoma

    • MALT lymphoma

    • Associated with H. pylori infection of the stomach

    • Treatment with surgery first and chemo if there’s a recurrence

  • Mycosis fungoides/Sezary syndrome

    • Skin lymphoma involving T cells

    • Called Sezary syndrome when the cells spill into the blood

    • Medium to large cells with convoluted (cerebriform) nuclei

  • Adult T cell leukemia/lymphoma

    • Seen in Japan and the Caribbean

    • Associated with HTLV-1 infection

    • See skin lesions, hepatosplenomegaly, generalized lymphadenopathy, and hypercalcemia

Category: Medical Subject Notes , Pathology Notes