ARTICLE
Myelodysplastic syndrome (MDS) is characterized by ineffective and dysplastic
hematopoiesis in one or more cell lines in the bone marrow. Cutaneous
lesions of MDS are usually separated into specific lesions and nonspecific
lesions, and whether atypical hematopoietic cells infiltrate the skin
or not. Early diagnosis of specific cutaneous lesions of MDS is important,
because these lesions can be the only clue to the diagnosis of MDS and
may precede acute transformation.
We report here on a patient with MDS, which was revealed by a specific
cutaneous lesion in the form of an ulcerated nodule.
Case report
An 86-year-old man visited our clinic in December 1991, because of an
ulcerated nodule on his left leg, with an erythematous border and fibrinous
exudate (Fig. 1). Histological
findings from this ulcerated nodule showed a dense infiltration of atypical
cells in the upper dermis through the subcutaneous tissue (Fig.
2). Neoplastic cells varied in size and were pleomorphic. Most
of the infiltrate was composed of large cells with round nuclei and prominent
nucleoli (Fig. 3). Immunohistochemical
studies were performed on paraffin sections. The atypical cells were negative
for CD3, L26, CD68, lysozyme, and glycopholin A. Periodic acid-Schiff
(PAS) stain did not demonstrate any micro-organisms. The blood count disclosed
pancytopenia (8.3 x 108 white blood cells/l with 8% blasts,
28 x 1011 red blood cells/l, 17 x 106 platlets/l).
A bone marrow smear showed hypercellularity with
25.7% blasts. These findings were compatible with the refractory anemia
with an excess of blasts in transformation (RAEB-T), typical of MDS. The
patient was then transferred to the hematology department to receive chemotherapy.
The ulcerated nodule decreased in size in response to combination chemotherapy
(methyl predonisolone, cytosine arabinoside, aclarubicine, etoposide),
but two months after visiting our out-patient clinic, acute transformation
occurred and the patient died.
Discussion
MDS comprises five pathological entities based on cytological features
and blast-cell numbers in bone marrow smears and peripheral blood; (i)
refractory anemia (RA), (ii) RA with ring sideroblasts (RARS), (iii) RA
with an excess of blasts (RAEB), (iv) chronic myelomonocytic leukemia
(CMML), (v) RAEB in transformation (RAEB-T) [1].
Transformation into acute myelogenous leukemia develops in 6 to 37%
of patients with MDS [2].
Various types of skin manifestations may occur during the course of
MDS. The non-specific lesions, which resemble Sweet's disease or pyoderma
gangrenosum, are characterized by an infiltration of neutrophils and are
known as neutrophilic dermatosis of MDS.
Specific lesions result from the dermal invasion
of malignant hematopoietic cells. Specific cutaneous lesions of MDS are
very rare [3]. Aractingi reported 18 out of 36 patients died in less than
3 months after the specific lesions developed [4]. The presentation of
the specific cutaneous lesion in MDS is closely related to acute transformation
and rapid deterioration.
The clinical appearance of specific lesions in MDS consists mainly of
papules, nodules, and tumors [4]. Our case was characterized by an ulcerated
nodule which mimicked pyoderma gangrenosum. A skin biopsy demonstrated
dense infiltrates of atypical hematopoietic cells, and immunohistochemical
studies showed that almost all infiltrated cells were negative for lymphocytic,
monocytic and erythrocytic markers. These findings supported the notion
that cells were myeloid origin.
Occasionally only a subjective symptom such as a skin lesion precedes
the diagnosis of MDS, as in our case. Peripheral blood examination before
biopsy was very helpful for the diagnosis.
REFERENCES
1. Bennet JM, Catvsky D, Daniel MT. Proposals for the classification
of the myelodysplastic syndromes. Br J Haematol 1982; 51: 189-99.
2. Sanz GF, Sanz MA, Vallespi T, Canizo MC, Torrabadella M, Garcia S
et al. Two regression models and scoring system for predicting
survival and planing treatment in myeloproliferative syndromes: investigation
of prognostic factors in 370 patients. Blood 1989; 74: 395-408.
3. Jacobs RH, Cornbleet MA, Vardiman JW, Larson RA, Le Beau MM, Rowley
JD. Prognostic implications of morphology and karyotype in primary myelodysplastic
syndromes. Blood 1986; 67(6): 1765-72.
4. Aractingi S, Bachmeyer C, Miclea JM, Verola O, Rousselot P, Dubertret
L, et al. Unusual specific cutaneous lesions in myelodysplastic
syndromes. J Am Acad Dermatol 1995; 33: 187-91.
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