Professional Documents
Culture Documents
000
41
Blackwell
Oxford,
International
IJD
0011-9059
1
UK
Science,
Journal
Ltd
2002
of Dermatology
Saskia A. Bouwhuis, MD, Rokea A. el-Azhary, MD, Marian T. McEvoy, MD, Lawrence E. Gibson,
MD, Thomas M. Habermann, MD, Thomas E. Witzig, MD, and Mark R. Pittelkow, MD
Abstract
Background 2-Chlorodeoxyadenosine (2-CdA), a purine adenosine analog, is safe and effective
chemotherapy for patients with hairy cell leukemia and low-grade lymphomas. Adverse effects
include neutropenia, lymphocytopenia, and infectious complications. Our objective was to
evaluate the efficacy of 2-CdA (2 6 seven-day cycles) in the treatment of late-stage, recalcitrant
Szary syndrome.
Methods Retrospective review of medical records of six patients with Szary syndrome who
had received 2-CdA cycles at Mayo Clinic, Rochester between March 1995 and March 2000.
Variables assessed from the records included improvement in global appearance, extent of
erythroderma, size of lymph nodes, pruritus, and leukocyte, lymphocyte, and absolute Szary
cell counts.
Results Two patients, both with stage III Szary syndrome, whose previous treatment consisted
of only two modalities, responded well to the treatment, with moderate to total clearing of
erythroderma and pruritus associated with a significant decrease in Szary cell counts. The other
four patients had only a partial response (one patient) or no response (three patients) to 2-CdA.
The mortality rate was 50%. All three patients died of Staphylococcus aureus sepsis. However,
only one patient was receiving 2-CdA treatment when he died. The other two patients died 8 and
9 weeks after the last 2-CdA cycle. This high mortality rate is attributed to infectious complications
after 2-CdA treatment in patients with recalcitrant disease.
Conclusion 2-Chlorodeoxyadenosine shows efficacy in stage III Szary syndrome, but it also
carries a substantial risk of septic complications and mortality. It can be used if no other suitable
alternatives are available. Caution should be exercised in all these patients regarding skin care and
avoidance of infections or sepsis.
Introduction
352
2-CdA, 2-Chlorodeoxyadenosine; CHOP, cyclophosphamide, doxorubicin, vincristine, and prednisone; ECP, extracorporeal photopheresis; PUVA, psoralen plus ultraviolet A.
*Died while receiving methotrexate therapy.
3
4
ECP, interferon alpha, PUVA, methotrexate
etretinate
4a
82 /F
6
3 year 6 month
2
3
ECP, PUVA, interferon alpha, systemic
corticosteroids
4b
77/M
5*
4 year 5 month
4b
60/M
4
1 year
1
2
6
12
3
3
6
3
No. of 2-CdA
cycles
Previous treatments
Duration
of disease
Stage
of disease
Age
(years)/sex
Results
Szary cell counts are known to fluctuate widely, this has not been
a problem at our institution. Serial Szary cell counts have been
found to be fairly consistent from day to day, particularly in the high
range (greater than 1000). Absolute Szary cell counts greater
than 500 are considered suspicious, and any count greater than
1000 is considered diagnostic for Szary syndrome. Szary cell
counts were recorded as an average of two different samples
taken on two consecutive days.
Follow up after
2-CdA (months)
1
2
3
Patient status
Alive
Alive
Dead
S. aureus sepsis, 8 weeks
after 3rd 2-CdA cycle
Dead
S. aureus sepsis, 4 weeks
after 2nd 2-CdA cycle
Dead
S. aureus sepsis, 9 weeks
after 3rd 2-CdA cycle
Alive
Patient
Bouwhuis et al.
353
354
Bouwhuis et al.
Patient
Global
response*
Erythroderma*
Pruritus*
Lymph
node size*
Leukocyte count,
total average 109 / l
Absolute neutrophil
count at nadir, 109 / l
Szary count
before 2-CdA
Latest absolute
Szary count
1
2
3
4
5
6
++++
++
++
++++
+
++
++++
+
++
++
NA
NA
++
8.7
7.5
8.5
7.3
15.6
10.3
1200
1400
600
800
1200
NA
10 965
1 824
8 870
7 350
13 578
7 239
0
41
2 965
7 950
14 616
5 979
Figure 1 Patient 1: (a) stage 3 Szary syndrome before 2-Chlorodeoxyadenosine treatment. (b) remission of Szary syndrome after
3 cycles of 2-Chlorodeoxyadenosine.
after her last 2-CdA cycle, the patient was still in remission. Patient 2, with stage 3 disease, had partial remission
(50%) after six cycles of 2-CdA. Her erythroderma and pruritus
improved moderately, and her absolute Szary cell count
decreased from 1824 to 41. Follow up at 1 year showed that
International Journal of Dermatology 2002, 41, 352 356
the absolute Szary count was still low at 100; her skin continued to show only mild erythroderma associated with mild
pruritus.
Patient 3, with stage 4a disease, had marked relief from severe
pruritus and severe erythroderma after the second of three
2002 The International Society of Dermatology
Bouwhuis et al.
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International Journal of Dermatology 2002, 41, 352 356
355
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Bouwhuis et al.