Case

Report

CUTANEUS LYMPHOMA IN
PATIENT WHO IS DIAGNOSED AS
MORBUS HANSEN AT BEGINNING
Hermawati Azikin
A.Fachruddin Benyamin
Department of Internal Medicine
Medical Faculty - Hasanuddin University

BACKGROUND

Background
Cutaneus lymphoma
Group of lymphoproliferative disorders: T
& B lymphocytes malignancy; part of
extranodal NHL
In the US: CTCL incidence (1973-2002)
6.4/1 million people/yr
Adult & elderly people: average age 40-60
yr
Children & young people: sporadic case

80% manifested as CTCL

 Cutaneous T-cell lymphoma

In 1979, National Cancer Institute (NCI)
introduced CTCL: group of lymphoproliferative
disorders characterized by the presence of
neoplastic T cells in the skin
Manifestations in the skin but in advanced
stage can involved hematology, lymph nodes
& visceral organs
Etiology:
Unknown
Chromosome aberration, especially in advanced
stage CTCL

 WHO- EORTC classified CTCL into 2

subtypes : indolent & aggressive type
Mycosis fungoides (MF)
Indolent subtype
The most common types of CTCL: 44%
Variant of MF (WHO/EORTC classification):
Sezary syndrome, folliculotropic MF,
granulomatous slack skin and pagetoid
reticulosis (Woringer-Kolopp disease)

 The diagnosis of MF: history of the

disease, clinical feature,
histomorphologic & cytomorphologic
feature, and cytogenetic examination
Management of CTCL
Early stage: topical therapy w/wo
interferon- or administration of oral
agents
Advanced stage: chemotherapy

CASE REPORT

History Taking
A male, 52 yo from Tual, admitted in Grestelina
hospital with complaints patches on the skin all over
the body
First experienced 3 yrs ago, the patches & plaques
raised on face, scalp, back, and chest initially, and
then raised neck, body, and extremities
In the course of illness:
fluid-filled lesions on the skin is reddish & fester
itchy patches & plaques especially when sweating
sometimes pain like puncture-pin

Hair becomes brittle, pts had a hair loss and so did

the eyebrows

 Subfebril fever during the disease

but when admitted to Grestelina
hospital no fever
No cough, no history of contact with
a cough person, no nausea,
decreased of appetite, decreased of
body weight. Defecation normal,
micturation normal.

Previous Illness History

3 yrs ago: pts went to Dermatologist in
Ambon & was diagnosed as Morbus
Hansen wo any information about skin
biopsy exam. He was given medication
(drugs & topical) for 2 months
History of contact with MH pts was denied,
job was staff of PLN
History drug user & free sex was denied.
No DM, family history with the same
disease was denied

 May 2013, the patient went to Ibnu Sina

Dermatologist in Makassar
Skin biopsy results: skin malignancies
Therapy: methotrexate, oral and topical symptomatic
therapy
Follow-up was carried out for 3 months with improved
of symptoms
No complete recovery, residence, & constraints to find
time for control discontinued methotrexate

Patches was recurred in all parts of the body

especially in chest, back & body fold
October 2013: pts was hospitalized in Grestelina
with a diagnosis of Cutaneous T-cell lymphoma/MF

Physical Exam
Conscious/Adequate
nutrition
Normal vital signs
Head
alopecia, red & black
patches on the scalp and
face, side of the head
look uneven shape,
madarosis

 Neck : JVP R-2 cmH2O, no

enlargement of lymph node,
no tumor mass
Thorax / Heart / Abdomen:
Normal
On the surface of the skin of
all body & extremities:
patches with hyperkeratosis
plaque, excoriation &
hypersquamation
Fingernails especially the
base of the finger was
damaged & dull

Laboratory Exam
Oktober, 22nd 2013
WBC : 13.840 /l
Hb : 11,1 gr/dl
PLT : 359.000 /l
AST/ALT : 20/23 U/L
Ur/Cr : 5/0,60
mg/dl
ESR : 70 mm

Oktober, 31st 2013

WBC : 8.610 /l
Hb : 11,4 g/dl
PLT : 207.000 /l
Ur/Cr : 19,0/0,7
mg/dl
ESR : 40+/75+ mm

Abdominal US
Mei, 31st 2013
Liver : enlarge
with flat surface,
homogen
echoparenchyme,
no nodular lesion
Impression :
hepatomegaly

Histopathology

Skin biopsy (July, 5th 2013)

Impression : cutaneus lymphoma (T-cell
lymphoma/mycosis fungoides)

 According to history taking, PE, &

supporting exam, this pts was
diagnosed as cutaneus T-cell
lymphoma with mycosis fungoides
type

DISCUSSION

DISCUSSION
The pts was initially diagnosed as Morbus Hansen
skin lesion: patches & thickening of the skin as a plaques
damaged of hair follicles: alopecia
pain like punctured-pin: neuropathy
WITHOUT skin exam/biopsy

In MH, skin manifestations:

diffuse erythema & thickening of the skin with infiltrates were
mainly found on the ears
face looks shiny & eyebrow usually be lost
hypopigmentation patches

Granulomatous MF is a rare type of CTCL with a clinical

picture that resembles lepromatous leprosy of MH

 Skin manifestations in early stage of MF

patchy hypopigmentation wo symptoms & lasts for
several years
no sensory defisit
skin lesions appear dry & dispigmentation multiple
ulcerated nodules
with anti-tuberculosis th/ in this pts no improvement,
which encourages further exam

After skin biopsy: CTCL

In the smear exam: no microorganisms
Histopathological exam: infiltrate of lymphocytes
in the dermis extending into the submucosa

WHO-EORTC Classification of
Cutaneous Lymphoma

 CTCL:
Slow-growing cutaneus lymphoma
Psoriasiform or diffuse erythroderma lesions
Atypical lymphocytes in the circulation
Early stage: resemble other skin diseases so
difficult to enforce

There are 2 types: MF & Sezary

syndrome
MF is the most common type of CTCL:
44%

Mycosis fungoides
Malignant lymphoma characterized by the expansion
of clon CD4 lymphocytes or T helper/T memory (CD45
+ RO) which are normally present on the skin
1st introduced in 1806 by Alibert-Bazin, Dermatologist:
severe disorder in the form of large tumors that
undergo necrotic which resemble mushrooms
The most common of CTCL (44%)
indolent type
3 stages: patches (atrophic or non-atrophic), plaques
sometimes accompanied by lymphadenopathy, tumor that is
susceptible to ulceration
Lesion distribution: asymmetric & most often on the back,
lower legs, groin, axilla & breast

Sezary syndrome
Clinical symptoms & signs: skin edema,
lymphadenopathy, hand & foot
hyperkeratosis, alopecia, nail
dystrophy, ectropion & organomegaly
Skin lesions: hypopigmentation patch,
erythematous with thickening of
patches
Aggressive type
5% of all cases of MF

 In this pts also presented alopecia

Alopecia was experienced before pts got
the treatment
Alopecia was found in 2.5% of pts with CTCL
Folliculotropic MF
Infiltration of atypical lymphocytes in the
epithelium of hair that inhibits hair growth
May reversible or persistant: topical steroids
and interferon

Alopecia can also occur secondary to

treatment

 Overall primary cutaneous lymphoma, 65% are T-cell

type, the most common is positive of CD4 immunotype
CTCL diagnosis is based on clinical symptoms and
course of the disease based on cyto &
histomorphologic feature
Blood tests (CBC) & blood smear
Liver function, uric acid & LDH
Flowcytometri
HIV & HTLV-1
Skin biopsy: atypical lymphocytes with lymph node biopsy
especially in pts with lymphadenopathy

Histopathological exam of MF pts seen a superficial

bandlike or lichenoid infiltrates, which is dominated by
lymphocytes & histiocytes.

Sezary syndrome diagnosis: > 1 of

the following criteria
Sezary cell count 1000 cells/uL
Abnormalities of the immune phenotype
T-cell clones in peripheral blood, as
indicated by molecular and cytogenetic
methods
Flowcytometri also used to determine the
differential diagnosis of precursor T-cell &
peripheral T-cell & NK-cell lymphoma

Examination of the skin biopsy

Staging is based on skin manifestations & extra

cutaneous manifestations:
Stage IA: patches / plaques <10% of skin surface area (T1)
Stage IB: patches / plaques> 10% of skin surface area (T2)
Stage II B: visible tumor (T3)
Stage III: thorough erythroderma
Stage IVA
A1: erythroderma and significant blood disorders
A2: lymph node biopsy showing atypical cells
Stage IVB: involvement of organs (liver, lung & bone marrow)
In this pts also conducted abdominal US hepatomegaly although
in examination no organomegaly was found
Plan for further exam to ensure the staging of disease
Based on physical examination & investigation in this pts, the
patient is diagnosed as cutaneous T-cell lymphoma, mycosis
fungoides staging IVB

 Management
Early stage: topical therapy w/wo interferon- or
other oral medications
Advanced stage: chemotherapy & current therapy

Some cytotoxic regimen had palliative function

but survival benefit is still questionable
In pts with advanced lesions, the choice of
therapy is systemic therapy
2nd line therapy for pts with skin lesions
Retinoids (bexarotene), IFN-, low-dose MTX, histone
deacetylase inhibitors (HDACi), or denileukin diftiox
Effective in refractory disease

 Low-dose MTX
oral every week, initial dose 20-30 mg/wks 60-70 mg/wks

HDACi: vorinostat
Can be well tolerated by the oral administration
Side effects: fatigue, light-moderate thrombocytopenia, elevated
creatinine, can be given together with PUVA, IFN-, MTX or in
combination with chemotherapy

Monoclonal antibodies: alemtuzumab

Targeting therapy against lymphocyte Ag
Systemic therapy for the provision of etoposide, vincristine, SF &
prednisone oral reported to respond to 80% at the advanced stage
CTCL or refractory MF

Transplantation is recommended for refractory younger pts

or advanced stage with the unresponsive to IFN-,
bexarotene, HDACi or denileukin diftitox
In this pts is planned to administrate chemotherapy, but the
patient is not willing to get this treatment
Pts received MTX with symptomatic th/ of oral & topical treatment

 In the early stages of MF lymphoma

cells is largely limited to the skin and
has a good response with only topical
therapy
In pts who only had partially respond
especially at an advanced stage, it
takes a systemic therapy

 Sandwich effect:
Rationalization of combination skin &
systemic th/ even in pts with limited skin
abnormality
Skin therapy works from the outside &
systemic therapy works from the inside
Mutually reinforce one another effect

 MF is a condition that cant be cured in most

pts except stage IA
Mortality & prognosis according to the stage of
disease at the time of first diagnosis
The involvement of lymph nodes, organs &
transformation to large T-cell lymphoma have
an aggressive clinical forms & usually die
Condition of this pts isnt experienced
significant improvement
Pts didnt get optimal th/ like systemic
chemotherapy

THANK YOU