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Dermatology Board Prep > DERM (18) Skin Growths > Cutaneous T-cell Lymphomas > Flashcards

Cutaneous T-cell Lymphomas Flashcards

1
Q

What percent of cutaneous lymphomas are mycosis fungoides?

A

50%

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2
Q

Epidemiology of mycosis fungoides?

A

Typical 50-60’s but can happen in lower aged patients (hypopigmented)

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3
Q

What is the general clinical progression of mycosis fungoides?

A

Progresses through patch, plaque, and tumor (in subset of patients) stages

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4
Q

What is the clinical morphology of that patch stage mycosis fungoides?

A

Irregular erythematous scaly patches occurring in non-sun-exposed/bathing suit distribution

Can be pruritic

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5
Q

What is the clinical morphology of mycosis fungoides?

A

Well-demarcated variably shaped violaceous to red-brown plaques, may be pruritic

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6
Q

What is the clinical morphology of tumor stage mycosis fungoides?

A

Rapidly enlarging nodules with frequent ulceration; arises in a background of patch and plaque lesions (otherwise unlikely to be MF)

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7
Q

What is the histology of patch stage mycosis fungoides?

A

Epidermotropic atypical lymphocytes (enlarged w/ cerebriform, hyperchromatic nuclei) predominantly in the epidermis in clusters (Pautrier’s microabscesses) and lined up at DEJ with clear halos surrounding the cells

Superficial dermal band- like/”lichenoid” lymphocytic infiltrate (predominantly reactive lymphocytes)

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8
Q

What is a key differentiator of epidermotropism from exocytosis?

A

You see more lymphocytes then you would expect for the degree of spongiosis

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9
Q

What is the histology of the plaque stage of mycosis fungoides?

A

More prominent epidermotropism w/ more atypical lymphocytes in the dense dermal band-like infiltrate

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10
Q

what is the histology of tumor stage mycosis fungoides?

A

Increased density and depth of dermal infiltrate of atypical lymphocytes with decreased/absent epidermotropism

  • Can also see a large cell transformation defined by >25% large cells(>4 times the size of a mature lymphocyte) +/− CD30 expression (often present, but not required for diagnosis)
  • Large cell transformation protends to poor prognosis
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11
Q

What is the definition of large cell transformation of mycosis fungoides?

A

>25% large cells(>4 times the size of a mature lymphocyte) +/− CD30 expression (often present, but not required for diagnosis)

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12
Q

What is the typical immunophenotype of mycosis fungoides T-cells?

A

Typical phenotype: CD3 +/CD4 + /CD8 - mature T-lymphocytes

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13
Q

What pan-t-cell markers are lost first and which ones are most specific?

A

CD7 loss most common (but least specific), CD5 and CD2 loss are less common but more specific

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14
Q

How useful are the t-cell receptor rearrangements in mycosis fungoides?

A

Can be helpful in diagnosis and should be sent, but be aware that inflammatory dermatoses, especially atopic dermatitis, can cause these to be falsely positive

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15
Q

In what patients is the hypopigmented form of mycosis fungoides usually seen in?

A

Darkly pigmented patients and children

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16
Q

What is the immunophenotype of the cells in hypopigmented MF?

A

CD8+

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17
Q

What are some histological differences between hypopigmented Mycosis fungoides and other things?

A

More interface changes (apoptotic keratinocytes and pigment incontinence)

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18
Q

Treatment for mycosis fungoides?

A

Varies by stage, but in general:

Patch/plaque: topical/intralesional steroids, nitrogen mustard, phototherapy, radiotherapy

  • Can add interferon-alpha or retinoids for progressive disease
  • Systemic chemotherapy is for advanced/rapidly progressive disease
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19
Q

What is the most common location of the folliculotrophic form of mycosis fungoides?

A

10% of patients with CTCL

Most common location is the head/neck and is associated with alopecia

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20
Q

What is the histology of the folliculotrophic mycosis fungoides?

A

Atypical infiltrates involve follicular epithelium + follicular mucinosis

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21
Q

What is the prognosis of folliculotrophic mycosis fungoides?

A

Increased depth of the infiltrate makes it more refractory to treatment which leads to worse prognosis (similar to tumor stage MF)

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22
Q

What is the clinical phenotype of pagetoid reticulosis?

A

Rare, progressive solitary psoriasiform plaque on
distal extremities

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23
Q

What is the histology of pagetoid reticulosis?

A

Very prominent epidermotropism in a pagetoid pattern

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24
Q

What is the prognosis of pagetoid reticulosis?

A

Good prognosis

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25
Q

What is the clinical morphology of granulomatous slack skin?

A

Extremely rare; sagging skin folds in the axilla/groin

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26
Q

What is the histology of granulomatous slack skin?

A

Granulomatous inflammation w/ multinucleated giant cells, atypical lymphocytes, and prominent elastophagocytosis

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27
Q

What is the prognosis of granulomatous slack skin?

A

Indolent course, often evolves into classic MF

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28
Q

What cancer can be associated with granulomatous slack skin?

A

Up to 30% develop Hodgkin’s lymphoma

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29
Q

What is the clinical presentation of Sézary syndrome?

A

Erythroderma (very pruritic!); lymphadenopathy. This is caused by sézary cells in the epidermis and is distinct from mycosis fungoides

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30
Q

How is Sézary syndrome diagnosed?

A

Circulating CD4+ neoplastic T-cells w/ an absolute count > 1000 cellcs/uL

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31
Q

Is the histology of Sézary syndrome helpful?

A

Not always, can look just like MF, need to check the blood

32
Q

What is the prognosis of Sézary syndrome?

A

Poor prognosis

33
Q

What cutaneous lymphoma is associated with HTLV-1 virus?

A

Adult T-cell leukemia/lymphoma

34
Q

What are the endemic areas of HTLV-1?

A

Japan, Caribbean, Central Africa

35
Q

What are the presenting symptoms of adult t-cell leukemia/lymphoma?

A

Leukemia, lymphadenopathy, organomegaly, hypercalcemia, and skin lesions

36
Q

What is the prognosis of adult T-cell leukemia/lymphoma?

A

Poor prognosis

37
Q

What is the histopathology of adult T-cell leukemia/lymphoma?

A

Resembles MF, but has characteristic “floret” or “clover-leaf” malignant T-cells

Immunophenotype: CD4+ /CD8- /CD25+

38
Q

What is the immunophenotype of adult T-cell leukemia/lymphoma?

A

CD4+ /CD8- /CD25+

39
Q

What is the CD30 status of lymphomatoid papulosis?

A

CD30+ (classified as an indolent lymphoma)

40
Q

What is the epidemiology of lymphomatoid papulosis?

A

Any age, but favors adults in 40s (vs PLEVA, which favors children)

41
Q

Clinical findings of lymphomatoid papulosis?

A

Multiple (10–20 lesions usually), recurrent, ulcerative, and red-brown papulonodules on trunk and extremities

The individual lesions self-resolve in 1 to 2 months and heal w/ atrophic varioliform scars

42
Q

How many types of lymphomatoid papulosis?

A

Types A,B,C,D,E,F and possibly G

43
Q

What is the histology of the type A type of lymphomatoid papulosis?

A

Wedge-shaped infiltrate with clusters of large, atypical, Reed-Sternberg-like CD30+ (Ki-1) lymphocytes

Increased mitotic activity and atypical mitoses, mixed inflammation (lymphocytes, eosinophils, and neutrophils)

Overlying ulceration and parakeratotic scale

44
Q

What is the most common form of lymphomatoid papulosis?

A

Type A (75%)

45
Q

What is the type B lymphomatoid papulosis?

A

Looks like patch/plaque MF

46
Q

What is the characteristic histology of lymphomatoid papulosis type C?

A

A dense pan-dermal infiltrate with sheets of CD30+ large lymphocytes is seen

-ALK-/EMA-

Histologically indistinguishable from ALCL and large cell transformation of tumor-stage MF → need clinical correlation

47
Q

What are the main CD30+ conditions?

A

MF in transformation (large cell), Arthropod assault/dermal hypersensitivity, lymphomatoid papulosis, hodgkin lymphoma, secondary anaplastic B-cell lymphoma, primary cutaneous anaplastic large cell lymphoma

48
Q

What is a difference in the immunophenotype between PLEVA and lymphomatoid papulosis?

A

CD38+

49
Q

What is the key histologic findings of type D lymphomatoid papulosis?

A

Epidermotropic CD8+/CD30+ cells; histologically looks like an aggressive epidermotropic T-cell lymphoma, but has much better prognosis

clinical correlation can help guide dx

50
Q

How often can t-cell receptor re-arrangements be seen in lymphomatoid papulosis?

A

50% of the time (not correlated w/ biologic behavior)

51
Q

Treatment of lymphomatoid papulosis?

A

Treatment is only for sx’s, there is no progression to secondary lymphomas. Methotrexate is reported to lead to dramatic improvement

52
Q

What can help distinguish PLEVA from type A lymphomatoid papulosis?

A

PLEVA is CD38+

Type A LyP = large CD30+ cells and dirty infiltrate w/ lots of eosinophils (not seen in PLEVA!) and neutrophils

53
Q

What is the association with lymphomatoid papulosis and lymphomas?

A

20% have an antecedent, concurrent, or subsequent lymphomas (MF>ALCL>Hodgkin’s lymphoma)

54
Q

What is the clinical appearance of primary cutaneous anaplastic large cell lymphoma?

A

Solitary (> multiple)

  • Ulcerated tumors up to 10 cm (larger than LyP); usually adults
  • Unlike LyP, lesions do not rapidly “come and go”
55
Q

What is the clinical course of Primary cutaneous anaplastic large cell lymphoma?

A

Frequently persists/relapses in skin, can rarely have nodal involvement

56
Q

What is the histology of Primary cutaneous anaplastic large cell lymphoma?

A

Sheets of large, atypical CD30+ lymphocytes making up more than 75% o the infiltrate

  • Most of the cells are CD4+
  • Lack ALK translocations (KEY) systemic ALCL has ALK translocations
  • EMA negative
57
Q

What is the prognosis of Primary cutaneous ALCL anaplastic large cell lymphoma?

A

Very good (90% 5-year survival)

58
Q

What is the immunophenotype of the cells in subcutaneous panniculitis-like T-cell lymphoma?

A
  • Lymphoma composed of CD4−/ CD8+/ CD56−/ TIA1+/ Granzyme B+ (cytotoxic) T-lymphocytes with α/β phenotype
  • There is also the “double-double negative (CD4-/CD8-)” aggressive from re-classified as γ/δ-delta T-cell lymphoma (universally fatal)
59
Q

What is the clinical presentation of Subcutaneous panniculitis-like T-cell lymphoma

A

Generalized subcutaneous nodules on legs and trunk

60
Q

What is the histology of the Subcutaneous panniculitis-like T-cell lymphoma

A

Subcutaneous lobular infiltrate of neoplastic T-cells that “rim” adipocytes; prominent necrotic debris and cytophagocytosis (“beanbag cells”)

Dacks interface changes at DEJ (vs γ/δ-delta T-cell lymphoma) and lacks nodular lymphoid aggregates and germinal center formation (vs lupus profundus)

61
Q

What is the prognosis of Subcutaneous panniculitis-like T-cell lymphoma

A

Good, 80-90% 5-year survival

62
Q

What is the immunophenotype of gamma/delta T-cell lymphoma?

A

CD4−/CD8− (“double negative”)

  • Expression of γ/δ T-cell receptor and cytotoxic markers (CD56+, TIA-1+, granzyme B+, and perforin+)
  • β-F1 negative (vs SPTCL, which is β-F1+) [this is a marker for the alpha/beta subtype which explains this]
63
Q

What is the clinical morphology of the primary cutaneous gamma/delta T-cell lymphoma?

A

Multiple eroded nodules and plaques + visceral involvement

64
Q

What is the histology of primary cutaneous gamma/delta T-cell lymphoma?

A

Dense dermal and subcutaneous lymphoid infiltrate w/ epidermotropism, lichenoid interface changes (major clue), vascular destruction, +/− fat rimming (mimicking SPTCL)

  • Lichenoid interface changes distinguish from SPTCL (never has epidermal involvement)
  • Lupus profundus is extremely hard to distinguish. The γ/δ stain is helpful and also lupus tends to have reactive lymphoid follicles (uncommon in
    γ/δ TCL
65
Q

What virus is associated with extranodal NK/T-cell lymphoma, nasal type?

A

EBV virus

66
Q

What is the clinical morphology of extranodal NK/T-cell lymphoma, nasal type?

A

Abrupt onset of ulcerated tumors, most commonly on nasal region

67
Q

Histology of extranodal NK/T-cell lymphoma, nasal type?

A

Variably sized neoplastic cells with prominent vascular destruction

68
Q

What is the immunophenotype of Extranodal NK/T-cell lymphoma, nasal type

A

CD2+/CD56+ and CD3+ (cytoplasmic, not surface –> due to picking up the early components of t-cell receptor like the epsilon subunit)

69
Q

Prognosis of Extranodal NK/T-cell lymphoma, nasal type

A

Usually fatal

70
Q

What is the clinical morphology of Aggressive epidermotropic cytotoxic (CD8+) T-cell lymphoma?

A

Eruptive ulcerated tumors with visceral involvement

71
Q

Histology of Aggressive epidermotropic cytotoxic (CD8+) T-cell lymphoma?

A

Malignant, cytotoxic, CD8+ infiltrate with prominent epidermotropism and angiodestruction

-Histologically indistinguishable from other epidermotropic CD8+ lymphomas (MF, pagetoid reticulosis, and type D LyP) so the distinction needs to be made clinically

72
Q

Prognosis of Aggressive epidermotropic cytotoxic (CD8+) T-cell lymphoma

A

Usually fatal

73
Q

What is the clinical phenotype of the Primary cutaneous CD4-positive small/ medium pleomorphic T-cell lymphoma?

A

Presents as a solitary plaque or nodule on the head/neck> upper trunk

74
Q

What is the prognosis of Primary cutaneous CD4-positive small/ medium pleomorphic T-cell lymphoma?

A

Excellent

75
Q

Histology of Primary cutaneous CD4-positive small/ medium pleomorphic T-cell lymphoma?

A

Histology: dense dermal/subcutaneous infiltrate of small to medium lymphocytes; minimal to no epidermotropism

-MF-like immunophenotype (CD4+/CD8−/CD30−)

Histology is the same as tumor stage MF, you need the clinical correlation [lacks preceding MF patches/plaques]

76
Q

What is the distinguishing factor between Primary cutaneous CD4-positive small/ medium pleomorphic T-cell lymphoma and tumor stage MF?

A

Lacks the preceding MF patches and plaques

DERM (18) Skin Growths (17 decks)

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