CTCL

Question 1

CTCL

Answer 1

cutaneous t cell lymphomas, a large group of t cell oriented conditions. Accounts for 80% of all primary cutaneous lymphomas.

Question 2

Dx of CTCL

Answer 2

Identify clonal T cells through a southern blot test or a PCR but it is difficult to dx.

Question 3

Classifying CTCL

Answer 3

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

Question 4

Mycosis Fungoides

Answer 4

Most common type of CTCL. m>f, ages 55-60. patch to plaque to tumor.

Question 5

Patho of MF

Answer 5

unknown, genetics v immune v environmental v long term infection.

Question 6

MF Clinical features

Answer 6

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.

Question 7

dermatopatholoy MF

Answer 7

Pautrier abscesses

Question 8

MF classification T - Skin

Answer 8

T1; Limited patches/plaques <10% BSA
T2: generalized, >10%
T3: Tumors
T4: Erythroderma

Question 9

MF Classification, N: lymph node involvement

Answer 9

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

Question 10

MF classification: M; Visceral involvement

Answer 10

m0: None involved, m1: involved

Question 11

MF classification: B blood

Answer 11

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

Question 12

What to include in assessment

Answer 12

physical exam, lymph node bx, skin bx, cbc, blood chemistry

Question 13

Treatment MF: Topicals

Answer 13

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

Question 14

Treatment MF: Radiation

Answer 14

localized radiation to specific lesions, total skin electron beam therapy, PUVA to slow growth of affected skin cells

Question 15

MF tx: Interferon

Answer 15

sq injection 3x a week, s/e: flu symptoms, alopecia, bone marrow suppression

Question 16

MF tx: Isotretinoin

Answer 16

s/e mucosal dryness, increased lipids

Question 17

MF Systemic Tx

Answer 17

Denileukin difitox - a fusion protein to neoplastic cells… s/e: capillary leak syndome, fever

Question 18

Classic MF begins as this

Answer 18

scaly patches that progress to plaques and eventually tumors, can take years

Question 19

Folliculotropic MF

Answer 19

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

Question 20

Tx Folliculotropic MF

Answer 20

remission is difficult. tx is PUVA, topical nitrogen mustard, interferon A, radiation, total skin electron beam

Question 21

Pagetoid Reticulosis

Answer 21

variant of MF. Slow progressing single psorasisform hyperkeratotic plaque or patch on an extremity. patho: large pagetoid cells. tx: local radiation or excision

Question 22

Granulomatous Slack Skin

Answer 22

very rare variant of MF. Males. Lax skin in axilla and groin. Tx: excision but will recurr, radiation possibly

Question 23

Adult T Cell Leukemia Lymphoma

Answer 23

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

Question 24

Primary Cutaneous Anaplastic Large Cell Lymphoma

Answer 24

single or grouped nodules that ulcerate. tx radiation, methotrexate, excision. severe with chemotherapy.

Question 25

Lymphomatoid Papulosis

Answer 25

chronic papulonecrotic lesions that self heal then recur, red brown color with central hemorrhage. tx: chemo or TSEB may cause temporary remissions

Question 26

Subacute Panniculitis T Cell Lymphoma

Answer 26

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

Question 27

tx Subacute Panniculitis T Cell Lymphoma

Answer 27

solitary lesions need local radiations, multiple need doxorubicin chemotherapy