Case Report

Two Case Reports on Cutaneous T-cell Lymphoma

Dr. Manoj More", Surg Capt Subhash Ranjan, N M,VSMb, Surg Capt S. C. Patra (Retd)', Surg Capt
Rahul Ray, VSMd, 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.

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 of which first 2 months
on Isoniazide+Rifampicin+Ethambutol
and next 8

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

Fig. 1: Photograph showing ulcerative and nodular lesions

over Right breast

months on Isoniazide+Rifampicin.
She was
investigated with MTB- PCR; found to be negative
hence A IT 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, Mumbai400005; 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-manojmore4@gmail.com.
58

Jour. Marine Medical Society, 2014, Vol. 16, No.1

lymphoma specifictherapywith marked improvement

and complete remission. He is on regular follow up.

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
cycles followedby Total skin electronbeam treatment
(TSEBT). Presently she is asymptomatic and is in
complete remission (CR).
Case No.2
This 47 year old male presented with multiple
itchyerythematoushyper pigmented nonscalyplaques
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 pustular lesions also developed over next
10 to 12 days. He had history of similar lesions 10
years back which were treated with some topical
ointments andthereafter subsided with leaving behind
hyper pigmented scar. His systemic examination was
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
along the line of pustularpsoriasiswith topicalsteroids
and antibiotics,but response was poor. His peripheral
blood smear was sent which showed Sezary cells.
Thereafter skin biopsy taken which confirmed the
diagnosis of mycosis fungo ides. Immunohistochemistry for CD3+, CD4+ was positive. He was
evaluated by the oncologist and started on cutaneous
Jour. Marine Medical Society, 2014, Vol. 16, No. I

Fig 3 : Hyperpigm ented mon-scaly plaques

Discussion
Recently the World Health Organization (WHO)
and European Organization for Research and
Treatment of Cancer Classification (EORTC) [11,
12] reached a consensus classification for cutaneous
lymphomas and revised by WHO in 2008. 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 clinical evidence 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-positiveblast cellsin the initialskin biopsy.

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
59

lymphoma usually presents in adults from 45 to 60

years of age, being more frequent in 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, appropriate diagnosis may 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.

:
I'

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
increased incidence of 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 diagnosis ofCD30+ large T-cell.
Histological
examination
shows
diffuse
nonepidermotropic infiltrates with cohesive sheets of
large CD30-positive tumor cells, oval or irregularly
shaped nuclei, prominent eosinophilic nucleoli, and
abundant cytoplasm. Additional common features are
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
60

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

positive in other CTCLs, especially tumor-stage MF
and subtypes ofLyP. EORTC sub classifies LyP 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 are utilized for the 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, spontaneous resolution may occur
over a period of weeks, 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 Tscell 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
Jour. Marine Medical Society, 2014, Vol. 16, No.1

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 sole modality.
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 of mortality 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

"_

Conflict(of interest
All authors have none to Declare.

Jour. Marine Medical Society, 2014, Vol. 16, No.1

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