CTCL Flashcards
CTCL
cutaneous t cell lymphomas, a large group of t cell oriented conditions. Accounts for 80% of all primary cutaneous lymphomas.
Dx of CTCL
Identify clonal T cells through a southern blot test or a PCR but it is difficult to dx.
Classifying CTCL
1) Utilize clinical signs, histology, and immunophenotypes to determine if it is mycosis fungoides or a subtype like Sezary Syndrome (these are 65% of cases of CTCL)
2) check for CD30 positive disorders (25% of cases)
3) remaining 10% are rare and need hem/onc to classify
Mycosis Fungoides
Most common type of CTCL. m>f, ages 55-60. patch to plaque to tumor.
Patho of MF
unknown, genetics v immune v environmental v long term infection.
MF Clinical features
fine scaly patch with mottled pigment changes early on, then advances to reddish, brown, indurated plaques. Increasing in size and shape, annual. Located on buttocks, trunk, extremities, May progress to tumor stage. Can become systemic and effect lymph nodes.
dermatopatholoy MF
Pautrier abscesses
MF classification T - Skin
T1; Limited patches/plaques <10% BSA
T2: generalized, >10%
T3: Tumors
T4: Erythroderma
MF Classification, N: lymph node involvement
N0: no lymph nodes involved
N1; enlarged lymph nodes, histologically uninvolved
N2: enlarged and involved
N3: enlarged and involved with the nodal architecture partially effaced
MF classification: M; Visceral involvement
m0: None involved, m1: involved
MF classification: B blood
b0: no circulatory sezary cells <5% of lymphocytes
b1: low tumor burden, >5% lymphocytes
b2: high tumor burden, >1k sezary cells plus a positive clone
What to include in assessment
physical exam, lymph node bx, skin bx, cbc, blood chemistry
Treatment MF: Topicals
need to stage patient first.
topical chemotherapy with nitrogen mustard (mechlorethamine) can lead to skin irritation, allergy, increased risk of skin cancers. Or BCNU (carmustine) which has same se, plus telangiectasias and myelosuppresion
Treatment MF: Radiation
localized radiation to specific lesions, total skin electron beam therapy, PUVA to slow growth of affected skin cells
MF tx: Interferon
sq injection 3x a week, s/e: flu symptoms, alopecia, bone marrow suppression
MF tx: Isotretinoin
s/e mucosal dryness, increased lipids
MF Systemic Tx
Denileukin difitox - a fusion protein to neoplastic cells… s/e: capillary leak syndome, fever
Classic MF begins as this
scaly patches that progress to plaques and eventually tumors, can take years
Folliculotropic MF
10% of all MF cases. Follicle/acne appearing papules, can be indurated. Head and necek usually involved. Alopecia in the eyebrows is a diagnostic clue with plaques in the ey be brows. Common secondary infectionsT
Tx Folliculotropic MF
remission is difficult. tx is PUVA, topical nitrogen mustard, interferon A, radiation, total skin electron beam
Pagetoid Reticulosis
variant of MF. Slow progressing single psorasisform hyperkeratotic plaque or patch on an extremity. patho: large pagetoid cells. tx: local radiation or excision
Granulomatous Slack Skin
very rare variant of MF. Males. Lax skin in axilla and groin. Tx: excision but will recurr, radiation possibly
Adult T Cell Leukemia Lymphoma
occurs in placed with human t cell lymphotropic virus type 1. Erythematous patches plaques and papules resembles MF. Tx: Interferon Alpha and Zidovudin, systemic chemotherapy
Primary Cutaneous Anaplastic Large Cell Lymphoma
single or grouped nodules that ulcerate. tx radiation, methotrexate, excision. severe with chemotherapy.
Lymphomatoid Papulosis
chronic papulonecrotic lesions that self heal then recur, red brown color with central hemorrhage. tx: chemo or TSEB may cause temporary remissions
Subacute Panniculitis T Cell Lymphoma
very rare, single or multiple nodules or plaques 1cm to 20cm, on extremities and trunk, ulceration may occur when resolved leaves areas of lipdystrophy. 50% get fever, weight loss, fatigue
tx Subacute Panniculitis T Cell Lymphoma
solitary lesions need local radiations, multiple need doxorubicin chemotherapy
