FastTest DCTCL...The overall incidence of CTCL is higher in men than women. a) TRUE b) FALSE 3. White people have a higher incidence of CTCL than non-white people. a) TRUE b) FALSE

FastTest

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... now test yourself

To ensure you have learned the key points that will improve your

patient care, read the authors’ questions below. Please refer back to

relevant sections of the book to address any areas of uncertainty.

1 Classification, epidemiology and etiology

1. Which of the following are true?a) CTCL is rare, with an incidence of < 1 per 100 000 persons

b) Sézary syndrome is the commonest form of CTCL

c) Indolent CTCLs generally have a good prognosis and prolonged

course

d) Aggressive CTCLs warrant systemic treatment

e) CTCL is not seen in pediatric populations

2. The overall incidence of CTCL is higher in men than women.

a) TRUE

b) FALSE

3. White people have a higher incidence of CTCL than non-white people.

a) TRUE

b) FALSE

4. Which of the following are known to be important in the pathogenesis of classic mycosis fungoides?

(a) Sunlight

(b) Smoking

(c) Alcohol intake

(d) Positive family history

(e) None of the above

5. In most cases, classic mycosis fungoides presents with which of the following?

a) Tumors on the head and neck

b) Erythroderma

c) Regressing nodules

d) Patches and plaques in a bathing trunk distribution

e) Nail dystrophy

2Clinical presentation and differential diagnosis

6. Hypopigmented mycosis fungoides is more common in older people and white skin.

a) TRUE

b) FALSE

7. Classic mycosis fungoides never presents in children.a) TRUE

b) FALSE

8. Comedones are a feature of folliculotropic mycosis fungoides.

a) TRUE

b) FALSE

9. Which of the following statements are correct? a) Sézary syndrome typically presents with a red scaly patch in the

bathing trunk area

b) Sézary syndrome does not involve the blood or lymph nodes

c) Sézary syndrome presents with erythroderma and atypical

lymphocytes in the blood

d) Sézary syndrome can cause extreme pruritus

e) Sézary syndrome has a poor prognosis and median survival of

3 years

10. Which of the following statements regarding lymphomatoid papulosis (LyP) are correct?

a) LyP is always associated with mycosis fungoides

b) Nodal involvement is a late event

c) There is an increased risk of a second lymphoma developing

d) The histological subtype does not affect outcome or treatment

e) A clinical history of self-resolving lesions is required to make the

diagnosis

11. How does lymphomatoid papulosis (LyP) usually present?a) As a slow-growing plaque on the head

b) As a slow-growing solitary tumor

c) As a single patch on the trunk

d) As symmetric patches on the hands and knees

e) With papules/nodules that enlarge and undergo necrosis before

healing spontaneously

12. Thickening of the facial skin folds (leonine facies) is a characteristic feature of primary cutaneous anaplastic CD30+ large cell lymphoma.

a) TRUE

b) FALSE

13. The differential diagnosis of mycosis fungoides may include:a) Discoid eczema

b) Atopic eczema

c) Psoriasis

d) Lichen planus

e) None of the above

14. Most commonly, clonal and atypical lymphocytes have which TCR phenotype?

a) a/d

b) b/g

c) g/d

d) a/b

3Pathology and diagnosis of mycosis fungoides and Sézary syndrome

15. Which histologic finding, although only present up to 20% of the time, tends to be most specific for mycosis fungoides?

a) Lichenoid infiltrate

b) Atypical appearing lymphocytes

c) Pautrier’s microabcesses

d) Lymphocytes lining up along the basement membrane

e) Langerhan’s cell microabcesses

16. In which stages of mycosis fungoides may one see lack of epidermotropism?

a) Tumor

b) Plaque

c) Patch

d) None of the above

17. A punch biopsy from a mildly verrucous plaque on the forehead of an otherwise healthy 7 year old female demonstrates follicular mucinosis. What is the best diagnosis on the basis of clinical presentation alone?

a) Lupus erythematosus

b) Folliculotropic mycosis fungoides

c) Follicular mucinosis

d) Plaque stage mycosis fungoides

e) None of the above

18. The T-cell subsets for mycosis fungoides and Sézary syndrome are distinct from each other.

a) TRUE

b) FALSE

19. Which subset of lymphocytes would you most commonly expect to find in a patient with mycosis fungoides?

a) Th2, CCR7, CD27

b) Th1, CLA, CCR4

c) Th2, CLA, CCR4

d) Th1, CCR7, CD27

20. A patient presents with thin, pink, scaled patches on the trunk, and bilaterally on the hips and thighs. Punch biopsy shows a patchy lichenoid infiltrate with scattered epidermotropic lymphocytes, diagnosed as mycosis fungoides. What is the most common immunophenotype associated with this diagnosis?

a. CD3+, CD4-, CD7+, CD8+, TIA-1+, CD45RA+, CD45RO-,

CD56-, EBER- and bF1

b. CD3+, CD4+, CD8-/+, CD30+

c. CD2+, CD3+, CD4+, CD5+, CD7-, CD8-/+, CD45RA-,

CD45RO+, and TCR-b+

d. CD3+/-, CD4+, CD8-, CD30+ with loss of CD5, CD7

e. CD3+, CD4-, CD8-/+, CD56+, TCR-g+, bF1-, EBER1-

21. An 87-year-old woman presents with erythroderma covering approximately 90% of her body, as well as alopecia, weight loss and night sweats. Her H&E biopsies show non-specific mild spongiosis, acanthosis and a mildly elevated CD4:CD8 ratio. What next steps would be most beneficial in obtaining a diagnosis?

a) Start prednisone, and repeat biopsies in 2–3 weeks

b) Do another biopsy for T-cell gene rearrangement

c) Perform flow cytometry to establish CD4, CD7, CD8 and CD26

subsets

d) Evaluate T-cell gene rearrangement in peripheral blood

22. A clinician sends in a patient sample only for T-cell gene rearrangement. On the form, the clinical information includes ‘Rule out CTCL’. The sample shows a monoclonal T-cell gene rearrangement and the pathologist diagnoses this patient with CTCL. Is this accurate?

23. A biopsy shows a diffuse dermal and upper subcutaneous infiltrate of neoplastic CD3+ CD4+ CD8- CD30- T cells with histiocytic granulomas composed of giant cells. Clinically, the patient has scattered thin, pink patches and multiple, thick scaly plaques on the hips, thighs and abdomen. The best clinicopathological diagnosis is:

a) Granulomatous slack skin

b) Mycosis fungoides, granulomatous variant

c) Interstitial granulomatous dermatitis

d) Subcutaneous panniculitis-like T cell lymphoma

e) None of the above

24. CD7 is a specific immunohistochemical marker for the diagnosis of:

a) Atopic dermatitis

b) Psoriasis

c) Mycosis fungoides

d) Tinea corporis

e) None of the above

25. In recent studies, Sézary syndrome has been associated with a T-follicular helper phenotype (PD-1+ CD10+ BCL-6+ CXCL-13). Which other hematopoietic disease has this phenotype?

a) Angioimmunoblastic T-cell lymphoma

b) Chronic myeloid leukemia

c) Blastic plasmacytoid dendritic cell neoplasm

d) Mycosis fungoides with large cell transformation

e) Cutaneous anaplastic large cell lymphoma

26. A patient with a 5-year history of stage IIA mycosis fungoides starts to develop larger tumors and infiltrative plaques. Clinically, large cell transformation (LCT) is suspected. Which findings are diagnostic of LCT and portend a worse prognosis?

a) CD3+ CD4+ CD30+ CD56- with approximately 10% large

transformed cells

b) CD3+ CD4+ CD8- CD30- CD56+ CD123+

c) CD3+ CD4+ CD30+ with approximately 30% large cells

d) CD3+ CD4+ CD30- CD56- with approximately 30% large cells

e) CD3+ CD4+ CD30+ with approximately 80% large cells in sheets

27. Two separate patients (A and B) present with slow-growing, indolent, multiple, crusted papules < 1cm in size, which wax and wane. Biopsy from patient A shows a wedge-shaped infiltrate of CD3+ CD4+ CD8-/+ CD30+ lymphocytes. Biopsy from patient B has an infiltrate composed of large, atypical, anaplastic CD3+ CD4+ CD30+ T cells present in nodules and sheets with a mixed inflammatory infiltrate. Which of the following statements are correct?

a) Patient A is likely to have lymphomatoid papulosis (LyP), while

patient B is likely to have primary cutaneous anaplastic large cell

lymphoma (PC-ALCL), the latter portending to a worse prognosis

b) Patient A and patient B are both likely to have LyP, and there are no

prognostic differences between the two subsets

c) Patient B may either have LyP PC-ALCL, and the histological

findings distinguish these diagnoses

d) Patient A has papular mycosis fungoides and the histological

findings are definitive

4Pathology and diagnosis of non-mycosis fungoides CTCL

28. A patient presents with rapidly evolving, ulcerated plaques and tumors on the trunk. Biopsy shows a dense infiltrate of CD3+ CD4- CD8- hyperchromatic, pleomorphic lymphocytes. The next steps in pathological workup should include:

a) T-cell gene rearrangement. If positive, the patient should be

diagnosed with CD4-/CD8- mycosis fungoides

b) TCR PCR on the peripheral blood and tissue. If positive, the patient

should be diagnosed with peripheral T-cell lymphoma (PTCL), NOS

c) Perform bF-1 stains. If negative, the patient should be diagnosed

with ld lymphoma

d) Perform bF-1 and TCR-g stains. If bF-1 is negative and TCR-g is

positive, the patient should be diagnosed with ld lymphoma

e) Perform CD56 immunophenotyping. If positive, the patient should

be diagnosed with extranodal NK/T-cell lymphoma, nasal type

29. A patient initially presents with small papules and thin plaques on the trunk and extremities that appear to self-resolve after approximately 8 weeks. A biopsy at that time shows a CD3+ CD4- CD8+ TIA-1+ epidermotropic infiltrate and the patient is diagnosed with lymphomatoid papulosis (LyP), type D. Four weeks later, he presents to his primary care provider with worsening joint pains, fatigue, night sweats and rapidly worsening skin findings, and is given a prednisone shot for his new joint pains. Upon follow-up, the patient has ulcerative infiltrative plaques on the trunk, +LAD, and +B symptoms. A repeat biopsy shows the same pattern and immunophenotypic profile of atypical T cells. What is the best diagnosis now?

a) Indolent CD8+ lymphoid proliferation of acral sites

b) Primary cutaneous aggressive epidermotropic CD8+ T-cell

lymphoma (Berti’s lymphoma)

c) Flare of LyP, type D

d) CD8+ mycosis fungoides

30. Match each of the types of lymphomatoid papulosis (LyP) – Type A, Type B, Type C, Type D, Type E, LyP with 6p25 – to the relevant diagnostic feature:

a) Angiotropism

b) CD8+ epidermotropism

c) DUSP/IRF4 rearrangements

d) Sheets of CD30+ cells

e) Reed–Sternberg-like cells

f) Commonly CD30-

31. Match each of the following non-mycosis fungoides CTCLs with the correct immunophenotype.

Primary cutaneous anaplastic large cell lymphoma (PC-ALCL)Subcutaneous panniculitis-like T-cell lymphoma (SPTCL)Primary cutaneous small/medium CD4+ lymphoproliferative

disorderPrimary cutaneous g/d lymphomaPrimary cutaneous aggressive epidermotropic CD8+ T-cell

lymphoma (Berti’s lymphoma)Extranodal NK-T cell lymphoma, nasal typeCutaneous adult T-cell leukemia/lymphoma (ATLL)Blastic plasmacytoid dendritic cell neoplasm

a) CD3+ CD4- CD8+ a/b+ TIA-1+

b) CD3+ CD4+ CD8- CD25+ FOX-P3+

c) CD3+ CD4- CD8-/+ CD56+ TCRg+ bF1- EBER1-

d) CD2+ CD56+ TIA-1+ EBER1+; CD3- CD4- CD5- CD7- CD8-

CD57- bF1- TCRg-

e) CD3+/- CD4+ CD8- CD30+ with loss of CD5, CD7; ALK1-

f) CD3+ CD4+ CD8- CD56+ CD123+ TCL1+

g) Epidermal CD3+ CD4- CD7+ CD8+ TIA1+ CD56- EBER- bF1+

h) CD3+ CD4+ CD8-, scattered CD20+

32. Complete the following sentence. Staging in mycosis fungoides …

a) is not necessary as all patients with MF have a normal life

expectancy

b) classifies patients into those with early stage disease (IA–IIA) or late

stage disease (IIB–IVB)

c) varies according to the depth (in millimeters) of the skin lesions

d) ranges from IA to IVB

e) is not necessary, as it only involves the skin

33. Complete the following sentence: When skin scoring mycosis fungoides and Sézary syndrome …

a) the percentage of body surface area covered by patches, plaques

and tumors should be included

b) never include disease on the buttocks

c) the palm approximates to 5% body surface area

d) use the palm as 0.5% body surface area to calculate areas of patch,

plaque and tumor

e) add the number of tumors over the body and multiply by 10

34. Which of the following is associated with a good prognosis in mycosis fungoides?

a) Poikiloderma

b) Young age

c) Co-existing lymphomatoid papulosis

d) Hypopigmented patches

e) All of the above

35. Complete the following sentence. Primary cutaneous large cell anaplastic lymphoma …

a) may undergo partial spontaneous remission

b) has an excellent survival with more than 90% of patients alive at

5 years

c) once treated, never recurs in the skin

d) presents with lymph node involvement

e) never metastasizes

36. Which of the following statements are correct?(a) Mycosis fungoides has an excellent prognosis and should be

managed by primary care physicians

(b) Primary cutaneous large cell anaplastic lymphoma is rare and

requires very different management to systemic disease

(c) Lymphomatoid papulosis may occur in association with other

CTCLs or systemic lymphoma

(d) Lymphomatoid papulosis (LyP) is rapidly fatal

(e) All CTCLs are Ebstein–Barr virus (EBV) positive

37. Which of the following statements are correct?a) The liver is never involved in CTCL

b) Cerebral metastasis may occur in CTCL

c) Most cases of CTCL are anaplastic lymphoma

d) Lymphomatoid papulosis has a poor prognosis

e) CTCLs are rarely cured

38. Cutaneous T-cell lymphoma should be managed by a multidisciplinary team, including:

a) Dermatologist

b) Oncologist

c) Hematologist

d) Pathologist

e) All of the above