20 - 119: CUTANEOUS LYMPHOMA Flashcards by Sharmaine Lozano

MC type of MF present indarker-skinned individuals

Hypopigmented MF

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Independent prognostic markers for survival, except

a. >60 years old
b. large cell transformation
c. increased LDH
d. decreased LDH

c. increased LDH

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Characterized by patches, plaques and hair loss within lesions

Folliculotrophic MF

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Solitary hyperkeratotic plaque on an extremitiy

Woringer-Kolopp disease – AKA pagetoid reticulosis

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Localized areas of bulky folding of the skin on the axillae and groins

Granulomatous slack skin

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T cell subtype present in Pagetoid reticulosis

CD8

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Sezary syndrome criteria except

a. >100/mm3 sezary cells in the blood – dapar >1000
b. CD4:CD8 ratio >10
c. loss of T cell antigens
d. loss of CD7 and CD26

a. >100/mm3 sezary cells in the blood – dapat >1000

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Lymphomatoid papulosis: wedged shape infiltrate with >75% large lymphocytes

Type A

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Lymphomatoid papulosis: angioinvasive/angiocentric type

Type E

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Lymphomatoid papulosis: mimics primary cutaneous aggressive epidermotropic CD8 cytotoxic TCL

Type D

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This is defined as neoplasm of clonal centrocytes and centroblasts with or without formation of follicles. Usually presents with solitary or grouped firm, painless, erythematous plaques and tumors.

Primary cutaneous follicular center lymphoma

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B-lymphocytes-derived malignancies that develop on the skin without extracutaneous involvement at the time of diagnosis and account for 20-25% of primary cutaneous lymphomas.

Primary cutaneous B-cell lymphoma

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Patients have small red-to-violaceous papules, plaques, or nodules. Seen most commonly on the upper extremities, or the trunk.

Extranodal marginal zone B-cell lymphoma

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Identified as a distinct clinical entity because of its perceived poor outcome compared with the indolent subtypes. Typically presents as solitary or clustered bluish erythematous plaques and tumors located on one or both legs.

Primary cutaneous diffuse large B-cell lymphoma, leg type

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First line therapy for primary cutaneous diffuse large B-cell lymphoma, Leg Type.

a. Rituximab
b. Cyclophosphamide
c. Hydroxydaunorubicin
d. Oncovin
e. Prednisolone
f. All
g. a and b

F. ALL

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T-cell population where clonal T cells in MF are commonly derived

tissue-resident memory cells (TRM)

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T-cell population where clonal T cells in Sezary syndrome are commonly derived

Central memory cells (TCM)

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HLA class II molecules that are significantly overrepresented in MF or sezary syndrome

HLA-DRB1∗11 and DQB1∗03

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virus associated with adult T-cell lymphoma/leukemia

human T-cell lymphotropic virus-1 [HTLV-1]

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virus associated with natural killer [NK]/T-cell lymphoma

Epstein-Barr virus [EBV]

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this virus is associated with CD30 lymphoproliferation and with immunosuppression.

EBV

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this bacteria may be responsible for chronic antigenic stimulation in CTCL

Staphylococcus aureus

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most common disease-related causes of death in CTCL

immunosuppression and opportunistic infections

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areas of predilection of tumor stage of MF

face and body folds

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erythroderma is defined as the involvement of how many % body surface area?

80%

Partial loss of these pan–T-cell antigens may be a feature of MF, but is not pathognomonic of the disease

CD7 and CD3

What is the 5-year survival rate of patients with MF limited to the skin?

80% to 100%

what is the 5-year survival rate of patients with lymph node involvement?

40%

independent prognostic markers for worse survival

* Stage IV, * age >60 years, * large-cell transformation, * increased lactate dehydrogenase

based on the criteria, how many points are required for the diagnosis of MF based on any combination of points from the clinical, histopathologic, molecular biologic, and immunopathologic criteria?

* presents with patches, plaques, and **unusual hair loss **within the lesions; occasionally, the disease may manifest with predominantly **papular lesions** * preferentially involves the head and neck region * affects mostly adults and is rarely observed in children and adolescents * occurrence of hair loss within the lesions, most conspicuous on the eyebrows, an intense pruritus, and secondary bacterial infection are common

Folliculotropic MF

* present with a solitary psoriasiform or hyperkeratotic patch or plaque, which is usually localized on the extremities and is slowly progressive * Unlike in classic MF, extracutaneous dissemination has not been observed. * shows more prominent epidermotropism and nuclear pleomorphism compared with unilesional MF, and more commonly shows a CD8+ phenotype * manifests more often as a **hyperkeratotic lesion.**

PAGETOID RETICULOSIS

* rare subtype of MF characterized by localized areas of bulky folding of skin, with a predilection for the axillae and groin * Light microscopy reveals a **dense granulomatous infiltrate** in the entire dermis * In addition to small, atypical cells with cerebriform nuclei, one finds macrophages and multinucleated giant cells and loss of elastic fibres. * The neoplastic cells express a CD3+CD4+CD8− phenotype.

GRANULOMATOUS SLACK SKIN

triad of Sezary Syndrome

1. diffuse erythroderma, 2. generalized lymphadenopathy, 3. circulating malignant T cells with cerebriform nuclei, so-called Sézary cells

Loss of expression of this CD is observed in majority of cases. It is also useful in the identification of Sézary cells.

Loss of **CD26 expression**

the diagnosis of Sézary syndrome requires an erythroderma with a positive T-cell clone in the peripheral blood associated with at least one B2 criterion, such as identification of more than how many Sézary cells/mm 3 in the blood.?

>1000

* characterized by recurrent, selfhealing crops of papules and nodules * chronic, recurrent, and self-healing papulonecrotic or papulonodular skin eruption * lesions typically involve the trunk and extremities, and lesions in various stages of evolution may be present concurrently

LYMPHOMATOID PAPULOSIS

* characterized by large tumor cells, of which the majority express the CD30 antigen, with no evidence or history of MF or other type of primary CTCL * characterized by the solitary or locoregional occurrence of reddish-to-brownish nodules and tumors, which frequently ulcerate

Primary cutaneous anaplastic large-cell lymphoma.

By definition, more than how mant % of the tumor cells in ALCL express CD30 in cohesive sheets?

75% ## Footnote ANAPLASTIC LARGE-CELL LYMPHOMA

In cases of solitary or localized skin lesions of ALCL, what is the treatment of choice?

excision or radiotherapy

In the case of extracutaneous dissemination of ALCL, what is the most frequently chosen option?

cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) *Brentuximab vedotin can also be regarded as a therapy of choice

This immunoconjugate is an anti-CD30 monoclonal antibody linked to monomethyl auristatin, a spindle-cell toxin that induces cell-cycle arrest

Brentuximab vedotin

* defined as a cytotoxic T-cell lymphoma characterized by the presence of primarily** subcutaneous infiltrates of small, medium, or large pleomorphic αβ T cells **and many macrophages that predominantly affect the **legs** and are occasionally complicated by a **hemophagocytic syndrome**

Subcutaneous panniculitis-like T-cell lymphoma

What are “B” symptoms?

weight loss, fever, and fatigue.

pivotal diagnostic marker for Subcutaneous panniculitis-like T-cell lymphoma

βF1 (TCR α/β)

The α/β type of the subcutaneous panniculitis-like T-cell lymphoma responds well to what medication?

systemic corticosteroids

* Immunophenotypically, the neoplastic cells express **CD56** and cytotoxic proteins (**TIA-1, granzyme B, perforin**), and are characteristically positive for EBV.This lymphoma is nearly always positive with EBV * What is the treatment of choice?

EXTRANODAL NATURAL KILLER/ T-CELL LYMPHOMA, NASAL TYPE * bone marrow transplantation may be the treatment of choice

best surrogate marker for survival

tumor burden

the most effective of all skin-directed therapies

Total skin electron beam

The 3 major biologic response modifiers (BRMs) used in the treatment of erythrodermic CTCLs

(a) oral retinoids, (b) ECP via an intravenous route, and (c) subcutaneous injections of IFN-α

A cure is defined as freedom from disease for how many years off all therapy?

8 years

this oral retinoid is used in CTCL

bexarotene

humanized monoclonal antibody that targets **CD52**, expressed on most T and B lymphocytes and NK cells.

Alemtuzumab

It is U.S. Food and Drug Administration approved for the treatment of relapsed-refractory Hodgkin lymphoma and **ALCL**

Brentuximab Vedotin ## Footnote anti-CD30 monoclonal antibody

MC side effect of brentuximab

induction of peripheral neuropathy

defucosylated **anti-CCR4 monoclonal antibody**

Mogamulizumab

a **new formulation of doxorubicin** in which the drug is **encapsulated in liposomes** and stabilized by the attachment of polyethylene glycol (ie, pegylation) to the liposomal surface, resulting in increased half-life and improved accumulation in tumor tissues.

Pegylated liposomal doxorubicin

, a **nucleoside analog of deoxycytidine** that inhibits DNA synthesis has shown activity against solid tumors as well as hematologic malignancies.

Gemcitabine

a novel **antifolate** with a **high affinity for the reduced folate carrier (RFC-1), **a novel mechanism of resistance when compared with methotrexate

Pralatrexate

Extranodal marginal zone lymphoma (EMZL)/primary cutaneous marginal zone lymphoma (PCMZL)

Primary cutaneous follicle center lymphoma (PCFCL)

Primary cutaneous diﬀuse large B-cell lymphoma, leg type

* neoplasm of clonal **centrocytes** (small and large cleaved follicle center cells) and **centroblasts** (large follicle center cells with prominent nucleoli) with or without formation of follicles. * A typical finding is the occurrence of lesions in a** circumscribed area of the head and neck region or the trunk,** but rarely on the legs

PRIMARY CUTANEOUS FOLLICULAR CENTER LYMPHOMA

in a subgroup of Extranodal marginal zone lymphoma (EMZL)/primary cutaneous marginal zone lymphoma (PCMZL), what bacteria is thought to have an etiologic role?

Borrelia burgdorferi