Coupon Accepted Successfully!


Mycosis Fungoides

Mycosis fungoides is synonymous with Cutaneous T cell lymphoma. It is the most common form of cutaneous lymphoma.
Clinical presentation and course:
  1. Mycosis fungoides has an indolent course.
  2. Most affected individuals have disease that remains localized to the skin for many years.
  3. It begins on the skin and may involve only the skin for years or decades
  4. Presentation is with localized or generalized erythematous / eczematous skin lesions.
  5. Skin lesion progress from 'patch' stage to 'plaque stage' to cutaneous 'tumors' stage.
  6. Metastasis occurs in advanced stages to:
1. To lymph nodes 2. Peripheral circulation
  Sézary syndrome (Erythroderma and Circulating tumor cells)
Seeding of the blood by melanoma tumor cells is accompanied by diffuse erythema and scaling of the entire body surface (erythroderma).
Sezary - Lutzner cells Pautrier Microabscesses
• Histological hall mark of
   Mycosis fungoides
• These are T helper cell (CD4 positive)
Sézary - Lutzner cells characteristically form band like aggregates within the superficial dermis and invade the epidermis as single cells or small clusters called Pautrier's Microabscesses.
Treatment (Mycosis fungoides is not easily amenable to treatment)
  1. The treatment of Mycosis fungoides is complex. Even early and aggressive treatment has not been proved to cure or prevent progression of the disease.
  2. Cure has been possible with radiotherapy only in rare patients with early stage mycosis fungoides.
  3. Most of the treatment for mycosis fungoides are palliative.
  4. Other T/T options are – steroid, topical nitrogen mustard & PUVA.

Note: Mycosis fungoides has an indolent course but it is not easily amenable to treatment.

Peripheral T-Cell Lymphoma, Unspecified

This is a 'wastebasket" diagnostic category.

Adult T- Cell Leukemia/Lymphoma (Atll)

  1. ATLL is a malignant T-cell disorder (CD4- T cells) due to HTLV-l infection that is found in Japan and the Caribbean. Q
  2. Clinical symptoms: skin lesions, hypercalcemia, enlarged lymph nodes, liver, and spleen. Q
  3. Microscopy hyperlobated “clover leaf” or flower cells and lymphocytes in the peripheral blood. Q

Anaplastic Large Cell Lymphoma

  1. Tumor of cytotoxic - T cells
  2. Large anaplastic cells with horse shoe shaped nuclei (hallmark cells)
  3. Tumor cells show AL k gene rearrangements on chromosome 2p23 in children on young adults.
  4. ALK gene rearrangements confer a good prognosis.
Large Granular Cell Lymphoma
  1. Also called as CD 8 Lymphocytosis, CD8+ T-CLL, g lympho proliferative disease. Q
  2. Tumor of CD8 + T-cells / NK cells Q
  3. Tumor Cells have abundant blue cytoplasm and coarse azurophilic granules
  4. C/F are due to neutropenia and anemia
  5. Many patients have hematological disorder like Felty syndrome.

Extra Nodal N. K/ T Cell Lymphomia

  1. Also called as lethal midlines granuloma
  2. Presents commonly as destructive mass of nasopharynx, rarely seen, testis etc
  3. Tumor of NK cell/ cytotoxic T. cells.
  4. Aggressive tumors'
Summary of the more common Lymphoid Neoplasms
Entity Salient Morphology Immunophenotype
Precursor B-cell lymphoblastic leukemia/lymphoma Lymphoblasts with irregular nuclear contours, condensed chromatin, small nucleoli, and scant agranular cytoplasm. TdT + immature B cells (CD19+, variable expression of other B cell markers)
Precursor T-cell leukemia/ lymphoma Identical to precursor B-cell lymphoblastic leukemia/lymphoma TdT+ immature T cells (CD2+, CD7+, variable expression of other T-cell markers)
Small lymphocytic lymphoma/ chronic lymphocytic leukemia Small resting lymphocytes mixed with variable numbers of larger activated cells; lymph nodes diffusely effaced CD5+ B cell expressing surface Ig
Follicular lymphoma Frequent small "cleaved" cells mixed with large cells; growth pattern is usually nodular (follicular). CD10+ BCL2+ mature B cells that express surface Ig
Mantle cell lymphoma Small to intermediate-sized irregular lymphocytes growing in a diffuse pattern CD5 – CD10 – mature B cells with surface Ig
Extranodal marginal zone lymphoma Variable cell size and differentiation; 40% show plasmacytic differentiation; B cells home to epithelium, creating "lymphoepithelial lesions" Mature B cells with variable expression of CD10 and surface Ig
Diffuse large B cell lymphoma Variable; most resemble large germinal center B cells; diffuse growth pattern Mature B cells with variable expression of CD10 and surface Ig
Burkitt lymphoma Intermediate-sized round lymphoid cells with several nucleoli; diffuse tissue involvement associated with apoptosis produces a "starry-sky" appearance Mature CD10+ B cells expressing surface Ig
Plasmacytoma/plasma cell myeloma Plasma cells in sheets, sometimes with prominent nucleoli or inclusions containing Ig Terminally differentiated plasma cells containing cytoplasmic Ig
Mycosis fungoides In most cases, small lymphoid cells with markedly convoluted nuclei; cells often infiltrate the epidermis (Pautrier microabscesses) CD4+ mature T cells
Peripheral T-cell lymphoma, not otherwise specified (NOS) Variable; usually a spectrum of small to large lymphoid cells with irregular nuclear contours Mature T-cell phenotype (CD3+)
Hodgkin lymphoma, nodular sclerosis type Lacunar Reed-Sternberg cell variants in a mixed inflammatory background; broad sclerotic bands of collagen usually also present CD15+, CD30+ Reed-Sternberg cells
Hodgkin lymphoma, mixed cellularity type Frequent classic Reed-Sternberg cells in a mixed inflammatory background CD15+, CD30+ Reed-Sternberg cells

Test Your Skills Now!
Take a Quiz now
Reviewer Name