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21 Cards in this Set

  • Front
  • Back

Most common type of Cutaneous T cell lymphoma?

Mycosis Fungoides

Pathogenesis of MF involves constitutive action of...




cytokine profile of MF?

JUNB and STAT3




TH2

What labs do we follow to assess disease activity?

What labs do we follow to assess disease activity?

Patch stage MF




follow serum thymus and activation regulated chemokine (TARC/CCL17) to assess disease activity

Pathology of MF?

superficial band like/lichenoid infiltrate of mainly lymphocytes




enlarged atypical cells with cerebriform and sometimes hyperchromatic nuclei




lymphocytes in epiD (epidermotropism)




colonize the basal layer as single cells with vacuolated pillow (lump of coal)




intraepidermal collections of lymphocytes (pautrier microabscess)




bare underbelly sign

CTCL: Immunophenotype

CD3+, CD4+, CD45RO+, CD8-, CD30-




variable CD2, CD5, CD7, often loss of expression




(if CD30+ happens to be expressed, it wil be MUM1+)

Large plaque parapsoriasis progresses to MF in what percent of cases?

10%

hydrochlorothiazide and MF?

may be a trigger... whack.

TMN staging for MF:




T1:


T2:


T3:


T4:




N0:


N1:


N2:


N3:


N4:




B0/1:


B2:




M0:


M1:

TMN staging for MF:


T1: patches, ppaules and plaques <10%


T2: patches, papules and plaques >10%


T3: 1 or more tumors


T4: erythema >80% BSA




N0: no nodes


N1: occasional to many lymphs in node


N2: aggregates of atypical lymphs in node


N3: complete effacement of LN


N4: no confirmatory histology




B0/1: <5% sezary cells


B2: >1000/uL sezary cells




M0: no visceral organs involved


M1: visceral organs involved

Skin directed therapeutics for MF include:

topical corticosteroids




nitrogen mustard




bexarotene




radiation




denileukin difitox

MOA of denileukin difitox?

CD25 is the target of fusion toxin (IL2)




fluid retention MC side effect

Side effects of bexarotene?

hyperTG


hypercholesterolemia


central hypothyroidism


leukopenia

MC location for follicular mucinosis?

Head and Neck (esp eyebrow)

Course?

Course?

Granulomatous slack skin




Indolent clinical course: 1/3 associated with Hodgkin's disease, may evolve to/from MF

Triad of Sezary Syndrome?

Aggressive and leukemic CTCL variant, only in adults:




Erythroderma




Lymphadenopathy




Tcell clone in blood (>1,000 sezary cells/uL, CD4/CD8 >10/1 on flow cytometry)

Usually MF is CD30-, if it is CD30+, what does this indicate?

CD30+ is associated with a more aggressive clinical course and poor survival

Chronic papulovesicular, papulopustular or hemorrhagic crusts with evolution to necrotic skin disease, mostly trunk and limbs --> usually asymptomatic and resolves over several weeks

Lymphomatoid papulosis

Primary cutaneous anaplastic large cell lymphoma prognosis?

10 year survival rate of 90%




represents 12% of cutaneous t cell lymphomas

In a biopsy, the presence of what suggests the skin lesion is probably a metastasis from an underlying systemic lymphoma?

ALK-1

HOTS mneumonic for adult T cell lymphoma?

HOTS




hypercalcemia, osteolytic bone lesions, t cell leukemia, skin lesions

What type of virus is HTLV? A/w what lymphoma?

retrovirus




a/w adult t cell lymphoma (0.01-0.02%)

MCC cause of death in extranodal NK/T cell lymphoma, nasal type?

lethal midline granuloma




EBV associated lymphoma, common in Asia, Cental/South America