ARTICLE
A 70-year-old Japanese woman was referred to us in August 1999 with a
2-month history of skin lesions. Examination revealed multiple dark-purple
to red, 10 to 40 mm, firm nodules on her left arm (Fig.
1) and back. Blood cell count was as follows: red blood cell count,
4.07 x 106/mm3; hemoglobin, 9.5 gm/dl; white blood
cell count, 5,360/mm3 with 3% stab cells, 54% segmented neutrophils,
39% lymphocytes, 2% monocytes, and 2% eosinophils; and platelets, 185,000/mm3.
No antibodies were detected to the human T cell leukemia virus type 1
or the human immunodeficiency virus. The multiphasic biochemistry series
were within normal limits. A biopsy specimen taken from a nodule on her
left forearm revealed a dense infiltrate of blastlike lymphoid cells in
the dermis with occasional mitotic figures and a grenz zone (Fig.
2). Formation of germinal center was not seen.
B-cell acute lymphoblastic leukemia with aleukemic
leukemia cutis
Immunohistochemical study on the skin biopsy specimen showed that the
lymphoid cells were positive for CD45 (leukocyte common antigen) and CD20
(L26) (Fig. 3), but negative for CD3, CD45RO (UCHL-1),
CD34, and CD68 (KP-1), suggesting the cells were in B-cell lineage. A
week after the skin biopsy, the bone marrow aspirate disclosed an 86%
infiltration of lymphoblasts with acute lymphoblastic leukemia (ALL) L2
morphology. Blood cell count at the same day was similar to the last one.
FACS analysis was not performed for economic reasons. Immunohistochemical
study on the bone marrow biopsy specimen showed the same results as on
the skin biopsy specimen. An overall diagnosis of B-cell ALL with aleukemic
leukemia cutis was made. However, because disseminated intravascular coaglation
(DIC), probably due to leukemia, suddenly occurred three weeks after the
first examination, the patient died of intracranial hemorrhage in September
1999.
Comments
Acute lymphoblastic leukemia (ALL) is classified into three classes
(L1 to L3) morphologically. Recently, several proposals for immunological
classification for ALL were put forward by some groups [1-3]. Pileri et
al. suggested that the CD20 molecule appeared to be restricted to
B-ALL [1]. According to their immunophenotyping of acute leukemia, a diagnosis
of B-ALL could be established in our case. Although the incidence of leukemia
cutis is highest in the monocytic leukemias [4], the marker of macrophage/monocyte,
CD68, was negative in our case.
Leukemia cutis has been recognized as a dissemination or proliferation
of leukemic cells in the skin [5]. It may occur concomitantly with systemic
leukemia. When it precedes peripheral blood or bone marrow manifestation
of leukemia, the term "aleukemic leukemia cutis" is used [6].
Aleukemic leukemia cutis (ALC) is a relatively rare form of leukemia
cutis, thus the number of patients is limited even after immunophenotyping
of leukemias was introduced. Zengin et al. reviewed 5 cases of
ALC with ALL [7], but the cases have not been classified immunologically.
Although Taniguchi et al. reported a case of ALC with T-ALL [8],
no case of ALC with B-ALL has been reported previously in the English
language literature. Immunophenotypic classification of leukemias will
play an important role in modifying therapeutic protocols on the basis
of prognostic evaluation [9]. Although leukemia cutis is considered as
a grave prognostic sign [4], it is not definitive because the number of
patients is limited [7]. Thus, in addition to clinical and pathological
information, immunophenotypic or immunohistochemical information about
ALC should be collected.
References
1. Pileri SA, Ascani S, Milani M, et al. Acute leukaemia
immunophenotyping in bone-marrow routine sections. Br J Haematol
1999; 105: 394-401.
2. Borowitz MJ, Bray R, Gascoyne R, et al. US-Canadian
Consensus Recomendations on the immunophenotypic analysis of hematologic
neoplasia by flow cytometry: data analysis and interpretation. Cytometry
1997; 30: 236-44.
3. Bene MC, Castoldi G, Knapp W, et al. Proposals for
the immunological classification of acute leukemias. Leukemia 1995;
9: 1783-6.
4. Ratnam KV, Khor CJL, Su WPD. Leukemia cutis. Dermatologic
Clinics 1994; 12: 419-31.
5. Su WPD, Buechner SA, Li CY. Clinicopathologic correlations
in leukemia cutis. J Am Acad Dermatol 1984; 11: 121-8.
6. Yoder FW, Schuen RL. Aleukemic leukemia cutis. Arch Dermatol
1976; 112: 367-9.
7. Zengin N, Kars A, Özisik Y, et al. Aleukemic leukemia
cutis with acute lymphoblastic leukemia. J Am Acad Dermatol 1998;
38: 620-1.
8. Taniguchi S, Hamada T, Kutsuna H, Ishii M. Lymphocytic aleukemic
leukemia cutis. J Am Acad Dermatol 1996; 35: 849-50.
9. Melnick SJ. Acute lymphoblastic leukemia. Clin Lab Med
1999; 19: 169-86.
|
|
|
|
Figure
1. Nodules on the left arm. |
|
|
|
|
|
Figure 2. A dense
infiltrate of blastlike lymphoid cells in the dermis (A) with
occasional mitotic figures and a grenz zone (B). |
|
|
|
|
|
Figure 3. Immunohistochemical
study showed that the lymphoid cells were positive for CD20 (L26). |
|
|
Printable version