HAEM5:Primary cutaneous marginal zone lymphoma: Difference between revisions
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Put your text here <span style="color:#0070C0">(''Instructions: Include associated hereditary conditions/syndromes that cause this entity or are caused by this entity.'') </span> | Put your text here <span style="color:#0070C0">(''Instructions: Include associated hereditary conditions/syndromes that cause this entity or are caused by this entity.'') </span> | ||
==Additional Information== | ==Additional Information== | ||
This disease is <u>defined/characterized</u> as detailed below: | This disease is <u>defined/characterized</u> as detailed below: | ||
* Primary cutaneous marginal zone lymphoma (PCMZL) is an indolent non-Hodgkin lymphoma arising in skin without evidence of extracutaneous disease at the time of diagnosis. The tumor is comprised of monotypic, CD5-negative, CD10-positive neoplastic small B-cells with monotypic plasma cells, and a variable number of reactive T-cells infiltrating the dermis, often forming follicles with reactive germinal centers. Clonal immunoglobulin rearrangement may be present, thus determining the subtype as class-switched versus non-class-switched heavy-chain immunophenotype. There must be no evidence of extracutaneous disease at the time of diagnosis and other cutaneous lymphomas must be excluded. | *Primary cutaneous marginal zone lymphoma (PCMZL) is an indolent non-Hodgkin lymphoma arising in skin without evidence of extracutaneous disease at the time of diagnosis. The tumor is comprised of monotypic, CD5-negative, CD10-positive neoplastic small B-cells with monotypic plasma cells, and a variable number of reactive T-cells infiltrating the dermis, often forming follicles with reactive germinal centers. Clonal immunoglobulin rearrangement may be present, thus determining the subtype as class-switched versus non-class-switched heavy-chain immunophenotype. There must be no evidence of extracutaneous disease at the time of diagnosis and other cutaneous lymphomas must be excluded. It has a favorable prognosis (5-year disease-specific survival rate >98%), recurrence is common, and 4% of patients will have extracutaneous spread, particularly in patients with longstanding multifocal disease. | ||
The <u>epidemiology/prevalence</u> of this disease is detailed below: | The <u>epidemiology/prevalence</u> of this disease is detailed below: | ||
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The <u>sites of involvement</u> of this disease are detailed below: | The <u>sites of involvement</u> of this disease are detailed below: | ||
* Skin (most commonly on the trunk and arms) | *Skin (most commonly on the trunk and arms) | ||
The <u>morphologic features</u> of this disease are detailed below: | The <u>morphologic features</u> of this disease are detailed below: | ||
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The <u>immunophenotype</u> of this disease is detailed below: | The <u>immunophenotype</u> of this disease is detailed below: | ||
* Positive (neoplastic B cells) - CD20, CD22, CD79a, BCL2 | *Positive (neoplastic B cells) - CD20, CD22, CD79a, BCL2 | ||
* Negative (neoplastic B cells) - CD5, CD10, BCL6, Cyclin D1 | *Negative (neoplastic B cells) - CD5, CD10, BCL6, Cyclin D1 | ||
*Positive (associated reactive germinal centers B cells) - BCL6 | *Positive (associated reactive germinal centers B cells) - BCL6 | ||