Papular mycosis fungoides: Six new cases and association with chronic lymphocytic leukemia

Table 2 Six additional patients with papular mycosis fungoides

Pt	ARG; Dur1	Lesions and Size (mm)	Distribution	Dermal Infiltrate	Epidermal Lymphocytes	Immunophenotype2	TCR-γ(method)	Course and Status (Duration FU)
1	68WM; 15 mo	Pa (1-2)	T, UE, UE	PA, VA, F, PC, Eos, LVC	Focal basilar Ep, Med CLs; No PMAs	ED: NS D: CD4 > CD8, CD30 10%	Pos Sk + Bd (SSCP)3	Controlled on prednisone; developed pancytopenia; DwD (99 mo)
2	47 WM; 19 yr	Pa (3-5)	H/N, T	A, PV, F, Eos, PC, MtF, LVC	Focal basilar Ep, Med-lg CLs; PMAs	ED: CD4+8-7-62L-30- D: CD4 90%, CD8 < 10%, CD7< 10%, CD62L < 1%, CD30 5%-10%	Pos Sk + Bd (DGGE)3	Poor or partial response to PUVA, MTX, isotretinoin, XRT, IFNα; DwD (171 mo)
3	57 WF; 5 yr	Pa (2-5)	LE, UE	P, A, Li, PV, CLs	Basilar Ep, CLs; No PMAs	ED: CD4+8-7+62L+30+/- 4 D: CD4 80%, CD8 20%-30%, CD7 40%, CD62L 50%, CD30 1%-2%	Neg (DGGE)	PUVA/NBUVB: CR; breast CA; RA, HT; A, NED (156 mo)
4	68 WM; 6 mo	Pa (2-8)	T, LE	A, Li, F, EE, Neu (v), CLs	Diffuse Ep, Med CLs; No PMAs	ED: CD4+/-8-7+62L-30- D: CD4 60%-70%, CD8 20%, CD7 70%, CD62L 70%, CD30 1%-2%	Pos (DGGE)	PUVA: CR ; no progression; AwD (210 mo)
5	58 WM; 2 mo	PaNd (2-14)	T, LE	Sp, Li, F, FM, CLs	Basilar Ep, Med-lg CLs; PMAs	Not available	Neg (SSCP)5	TopHN2: CR; No progression; A, NED (171 mo)
6	81 WM; 15 mo	PaNd (5-15)	T, LE	Li, F, CLs, MtF	Diffuse Ep, Med-lg CLs; PMAs	ED: CD4+8-7-62L-30- D: CD4 99%, CD8 1%, CD7 10%, CD62L 99%, CD30 20%	Neg (DGGE)3,5	PUVA: PR; DwD/MI (12 mo)

¹Duration: Time from onset of disease to evaluation;

²Estimated percentage of positively labeled cells in dermal infiltrate (frozen sections). CD2, CD3, and CD5 expressed by all cases (data not shown);

³Clonal B-cell population detected by flow cytometry for patients 1, 2 and 6 and confirmed by PCR for patients 1 and 2 (see text for details);

⁴30% of epidermotropic T cells expressed CD30;

⁵Blood sample also negative for T cell clone. Pt: Patient; ARG: Age, race, gender; TCR-γ: T-cell receptor gamma chain rearrangement; FU: Follow up; Pa: Papule; Nd: Nodule; T: Trunk; UE: Upper extremity; LE: Lower extremity; A: Acanthosis; Sp: Spongiosis; P: Parakeratosis; VA: Vacuolar alteration; Ep: Epidermotropism; Li: Lichenoid (band-like); PV: Perivascular; PA: Periadnexal; PDE: Papillary dermal edema; F: Fibrosis; FM: Follicular mucinosis; Neu (v): Neutrophils in vessels; PC: Plasma cells; Eos: Eosinophils; EE: Extravasated erythrocytes; CL: Lymphocytes with cerebriform or infolded nuclei (mycosis cells); MtF: Mitotic figures; LVC: Lymphocytes with vesiculated nuclei and prominent nucleoli; PMA: Pautrier microabscess; Sm-med: CLs with small-medium sized nuclei (5 to 7 µm in diameter); Med-lg: CLs with medium-large sized nuclei (7 to 9 µm in diameter); ED: Epidermis; D: Dermis; >: Greater than; <: Less than; NS: Not stated; Sk: Skin; Bd: Blood; DGGE: Denaturing gradient gel electrophoresis; SSCP: Single-stranded conformation polymorphism; MTX: Methotrexate; XRT: Local field radiation therapy, IFNα: Interferon alfa; PUVA: Methoxsalen-ultraviolet A photochemotherapy; NBUVB: Narrow band ultraviolet B phototherapy; TopHN2: Topical mechlorethamine; CA: Carcinoma; RA: Rheumatoid arthritis; HT: Hashimoto’s thyroiditis; MI: Mycocardial infarction; AwD: Alive with active disease; DwD: Dead with active disease; A: NED, alive, no evidence disease; LTF: Lost to follow up.