Two Case Reports on Cutaneous T Cell Lymphoma. Journal of Marine Medical Society 2014 Vol 16 No. 1

8/10/2019 Two Case Reports on Cutaneous T Cell Lymphoma. Journal of Marine Medical Society 2014 Vol 16 No. 1

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Case Report

Two Case Reports on Cutaneous T-cell Lymphoma

Dr. Manoj More , Surg Capt Subhash Ranjan,

N M,VSM

b

Surg Capt S. C. Patra

Retd) ,

Surg Capt

Rahul Ray,

VSM

d

Surg Cdr Hari

Mukundarr,

Surg Crude Naveen

Chawla'

Background

Cutane~us T-cell lymphoma (CTCL) has a variable

presentation and comprises a broad diagnostic group.

Histologic and immunophenotypic confirmation is

needed to establish a precise diagnosis. Once the

categorization is determined, prognosis and

therapeutic algorithms unfold. Primary cutaneous,

CD-30+, Anaplastic large T-cell lymphoma

represents an indolent form of CTCL that often

spontaneously involutes. CTCL is a broad diagnosis

encompassing a spectrum of disease. The European

Organization for Research and Treatment of Cancer

(EORTC) Cutaneous Lymphoma Project recognizes

mycosis fungoides (MF), cutaneous CD30-positive

large-cell lymphoma, and lymphomatoid papulosis

as CTCLs with indolent clinical behavior [1]. Clinical

presentation and immunohistochemistry together are

needed to determine the subtype of CTCL, which

guides proper management.

Clinical Synopsis

Case No.1

This 47 year old lady presented with history of

swelling over left armpit and breast since one and

half year, burning pain over left breast since 2 months

and weight-loss since 4 months. Comorbidity:

Hypothyroidism. Considering axillary

lymphadenopathy, strongly positive Monteux test

with history of weight loss of 6 kg 54 to 48 kg in 4

months, she was started on antituberculous therapy

w.e.f. Nov 2010. In July 2011 (after 8 months), she

came to INHS ASVINI for increase in number of

swellings in left axilla inspite of treatment. She was

evaluated with Ultrasound left axilla which showed

large lymph nodes with no evidence of necrosis or

caseation. Fine needle aspiration showed reactive

lymphadenitis. Screening Mammography showed

normal study. Ultrasonography of abdomen revealed

grade 2 fatty liver. Lymph node biopsy from axilla

done twice revealed only reactive lymphadenitis. She

was on antituberculous therapy ofwhich first 2 months

on Isoniazide+Rifampicin+Ethambutol and next 8

Fig. Photograph showing ulcerative and nodular lesions

overRight breast

months on Isoniazide+Rifampicin. She was

investigated with MTB- PCR; found to be negative

hence AIT was stopped. Of late, she developed

erythematous lesions over left breast which was itchy

painful and gradually increasing in size to form an

ulcer. Similar lesions developed over axilla and

breasts over a period of 6 weeks. For next 15 days

she had increasing burning pain disturbing her sleep

and appearance of similar lesion on trunk made her

Resident, Department of Medicine; 'Senior Advisor (Medicine Oncology), INHS ASVINI, Colaba, Mumbai-

400005; cProfessor

&

Head, Department of Surgery, ESI Post Graduate Institute of Medical Science

&

Research, Mahatma Gandhi Memorial Hospital, Parel, Mumbai - 400012. Senior Advisor Dermatology,

eClassifIed Specialist Radiation Oncology, Consulrant Pathology, INHS ASVINI Colaba, Mumbai - 400 005.

Corresponding author: [email protected].

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Fig 2 : Cutaneous T cell lymphoma mycosis fungoides

High power view.

to see the dermatologist. She was started on topical

Flutibact (fluticasone propionate 0.005 w/w,

mupirocin 2 w/w.) ointment and skin biopsy was

taken. Report of biopsy showed nonnal epidermis,

infiltrate of neutrophils and foci of micro abscess.

Review of biopsy blocks at higher Cancer Centre

showed anaplastic cutaneous T cell lymphoma.

Immunohistochemistry for CD 30 was positive. She

was evaluated by oncologist and started on

chemotherapy, CHEOP (Cyclophosphamide,

Doxorubicin HCI, Etoposide, Vincristine,

Prednisolone with GCSF protection) protocol x 6

cyclesfollowedby Total skin electronbeamtreatment

(TSEBT). Presently she is asymptomatic and is in

complete remission (CR).

Case No.2

This 47 year old male presented with multiple

itchyerythematoushyperpigmentednonscalyplaques

of varying size ranging from 5 mm to 8 em involving

all body but predominantly over back, chest,

abdomen and flexor and extensor aspect of upper

limbs. Some pustularlesionsalsodevelopedovernext

10 to 12 days. He had history of similar lesions 10

years back which were treated with some topical

ointmentsandthereafter subsidedwith leavingbehind

hyper pigmented scar.His systemic examinationwas

unremarkable. On evaluation he had normal

hemogram and biochemical tests, negative for

HBsAg, anti HCV, HIV ELIZA and VDRL.

Considering pustular lesion he was initially treated

alongthe line ofpustularpsoriasiswith topicalsteroids

and antibiotics,but responsewas poor. Hisperipheral

blood smear was sent which showed Sezary cells.

Thereafter skin biopsy taken which confirmed the

diagnosis of mycosis fungo ides. Immunohis-

tochemistry for CD3+, CD4+ was positive. He was

evaluatedby the oncologist and startedon cutaneous

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lymphomaspecifictherapywithmarked improvement

and complete remission. He is on regular follow up.

Fig

3 : Hyperpigm

ented mon-scaly plaques

Discussion

Recently theWorld HealthOrganization(WHO)

and European Organization for Research and

Treatment of Cancer Classification (EORTC) [11,

12] reached a consensus classification for cutaneous

lymphomas andrevisedbyWHO in2008. Cutaneous

T-cell lymphomas are subdivided into the following

classifications.

Mycosis fungoides is the most common type of

CTCL and accounts for almost 50 of all primary

cutaneous lymphomas. The second most common

group of CTCL is primary cutaneous CD30+

lymphoproliferative disorders. Primary cutaneous,

CD30-positive, large-cell lymphoma represents

about 10 percent of all cases ofCTCL [1]. Primary

cutaneous CD30-positive large cell lymphoma is

defmed according to the following criteria [2, 3] :

1. No clinicalevidence oflymphomatoid papulosis

2. No previous or concurrent lymphomatoid

papulosis, mycosis fungo ides, or other

(cutaneous) lymphoma

3. No extracutaneous localization at presentation

4. Predominance (>75 ) of large clusters of

CD30-positiveblastcellsin the initialskinbiopsy.

CD30+, cutaneous large T-cell lymphoma and

lymphomatoid papulosis (LyP) are considered by

some to be within the same spectrum of low-grade,

cutaneous,anaplastic,large-celllymphomas (ALCL).

Clinical distinction can be argued between the two.

Because both portray similar histology with atypical

CD30+ T-cells, clinical appearance is stressed by

EORTC to distinguish between the diagnoses and to

delineate treatment. CD30+ cutaneous T-cell

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lymphoma usually presents in adults from 45 to 60

years of age, beingmore frequentin males. It presents

as one to several localized nodules or tumors with

ulceration. Twenty percent of cases are multifocal.

Plaques are greater than 1 em in most cases (77 )

[4]. Trunk and extremities are most commonly

involved. Presentation may be variable, being

mistaken for other skin disorders such as adult-onset

eczema, pyoderma gangrenosum,morphea, localized

scleroderma, or squamous cell carcinoma [5].

Consequently, appropriatediagnosismay be delayed.

Lymphomatoid papulosis is a chronic disease that is

widespread with recurring crops of numerous

papules, nodules and plaques. Lesions evolve and

may necrose and ulcerate.

The onset ofLyP is earlier, typically the third or

fourth decade, although it rarely presents in children.

Females are more commonly affected. There is an

increasedincidenceof associatedlymphopro1iferative

disorders with LyP, such as mycosis fungoides and

Hodgkin lymphoma. Reportedly, 5-10 percent may

evolve to malignant lymphoma [6]. Spontaneous

resolution with episodic recurrence is common.

Despite the anaplastic nature, primary cutaneous,

CD30+, anaplastic large T-cell lymphomas are no

more aggressive than non-anaplastic type ofCD30+

T-cell lymphomas. The EORTC combines these two

histologicallydistinct,but clinicallysimilar,cutaneous

lymphomas into the diagnosisofCD30+ large T-cell.

Histological examination shows diffuse

nonepidermotropic infiltrateswith cohesive sheets of

large CD30-positive tumor cells, oval or irregularly

shaped nuclei, prominent eosinophilic nucleoli, and

abundantcytoplasm.Additionalcommon featuresare

epidermal ulceration (63 ), prominent vascular

proliferation (60 ), pseudo epitheliomatous

hyperplasia(Sf ), tumor necrosis (55 ), and

vascular infiltration by neoplastic cells (44 )[4].

Occasionally (20-25) Reed-Sternberg-like

pleomorphic or immunoblastic cells are present [7].

The mitotic index is high. Reactive lymphocytes and

plasma cells rarely present at the periphery.

hrununohistochemistry is essential in determination

of CTCL subtypes and in the differentiation between

primary and secondary disease. A predominance of

greater than 75 percent CD30+ T-cells must be

present for diagnosis as CD30+ large T-cell

lymphoma. Neoplastic cells express an activated

CD4-positive T-cell phenotype with variable loss of

CD2, CD3, and CD5 [7]. CD30+ cells may be

positive in other CTCLs, especially tumor-stage MF

and subtypes ofLyP. EORTC sub classifiesLyP into

histological subtypes, A, B, and C. Types A and C

both express CD30+ cells. Histologically, Type C

may resemble CD30+large T-cell lymphoma [6].

Once clinical appearance, histology, and

immunohistochemistry support the diagnosis of

CD30+ large T cell lymphoma, a thorough

examination with attention to skin, lymph nodes,

spleen, and liver is necessary. Chest radiography,

computed tomography of abdomen and pelvis, bone

marrow biopsy, lymph node analysis, and complete

blood count with smears can further assist in

identification and staging of secondary cutaneous

systemiclymphoma.Multidisciplinarycare is optimal.

Twenty-five percent of CD30+ CTCLs have

lymph node involvement at presentation and 12

percent are secondary cutaneous lesions [3, 8]. A

multitude of therapies areutilized forthe spectrum of

cutaneous T-cell lymphomas. Treatment varies with

the diagnostic category and scope of disease. Partial

or complete remission occurs with 42 percent of

CD.30+CTCLs [8].Relapse occurs in approximately

40 percent of patients despite treatment [8]. Extra

cutaneous disease occurs in 10-25 percent of

CD30+lymphomas despite treatment [2, 8]. Local

radiotherapy or surgical excision is effective for one

to several CTCL plaques, nodules, or tumors. If the

neoplasm relapses,spontaneousresolutionmay occur

over a period ofweeks, otherwise treatment may be

repeated. Treatment of multifocal disease is

challenging. Subcutaneous or oral methotrexate may

reduce generalized disease but does not prevent

progression to more aggressive lymphoma [9].

Chemotherapy should be reserved for patients with

more generalized cutaneous disease, those with

increased risk of systemic disease, and those who

develop systemic disease [3]. Multi-agent systemic

chemotherapy, compared to single agent, does not

result in a higher cure rate nor prevent future relapses

[2, 8]. Overall prognosis for primary cutaneous

CD30-positiveanaplastic large s ell lymphoma is

excellent. Total skin electron beam treatment

(TSEBT) may be used in patients with more

disseminated cutaneous disease. It is a treatment in

which ionizing radiation is administered to the entire

skin surface penetrating to the dermis. The standard

total dose is 36 Gy delivered with electrons of at

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least 4 MeV energy and fractionated over 8-10

weeks. Reported complete remission rates range

from 40 to 98 among patients with Tl and T2

stage; however, relapse rates are high when used as

the solemodality.

Nearly all patients developed skin-related side

effects including erythema, telangiectasia, and

systemic therapies [11]. However, multifocal disease

at presentation has a two-fold increased chance to

acquire extra cutaneous disease and a four-fold

increased chance ofmortality from the lymphoma [3,

8]. Anatomic site, size, cytology, and additional

immunologic markers have not been shown to

influence clinical behavior [3, 4]. Spontaneous

regression and age less than 60 years are associated

with a favorable prognosis [10]. Overall, primary

cutaneous CD30+ large T-cell lymphoma survival at

5 and 10 years is 95 percent [8]. Because a risk for

systemic progression exists, albeit low, longitudinal

observation is strongly recommended. Our patients

had relatively large CD30+ plaques. Clinical

presentation with immunohistological confmnation

supported the diagnosis as CD30+ large T-cell

lymphoma and Mycosis fungoides (MF). Although

known to spontaneously regress, chemotherapy was

implemented because of the low risk of systemic

spread and subsequent EBRT is also been planned.

These cases have been presented for review of

CD30+ large T-cell tumors.

How to cite the article

More M, Ranjan S, Patra S C, Ray R, Mukundan H, Chawla

N, Two Case Reports on Cutaneous T-cell Lymphoma.J

Marine

Medical Society

2014, 16 (1) : 58-61. .

Source of support

Nil _

Conflictof interest

All authors have none to Declare.

Jour Marine Medical Society 2014 Vol

16,

No 1

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Two Case Reports on Cutaneous T Cell Lymphoma. Journal of Marine Medical Society 2014 Vol 16 No. 1