378
tional stabilization of the protein which may also
abolish its function; c) overexpression of wild-type
protein in response to spontaneous genetic errors
occurring at a higher frequency in neoplastic tissue;
d) the effect of antigen retrieval techniques which can
alter detection thresholds; e) mutations occur out-
side the exons studied.
Several repo rts have described that p53 alteratio ns
are no t o bserved in mo re benign MDS cases.
2-6
How-
ever, this study, in keeping with a study do ne by Kita-
gawa et al.
9
revealed two RA cases sho wing p53 o ver-
expression suggesting that p53 abnormality may not
be a terminal genetic event during leukemia develop-
ment. To the best of our knowledge only one other
study has described p53 alteratio n in the RA phase.
10
The presence of DNA from normal cells in less
advanced subtypes is likely to affect the sensitivity o f
the mutation detection and may underestimate the
rate o f p53 mutatio n in RA phase.
Taking into account the shorter interval between
RA phase and progression, p53 overexpression may
have contributed to the pathogenetic process in the
progression of MDS in our cases. However, addi-
tional and more extended studies are necessary to
determine the genetic basis for this immunoreactivi-
ty and to clarify the prognostic value of such find-
ings.
Sílvia M.M. Magalhães, Larissa P. Ponte,
Francisco Valdeci A. Ferreira, Francisco Dário Rocha Filho
Hematology and Hemotherapy Center of Ceará – HEMOCE,
Department of Clinical Medicine, Department of Pathology,
Labtech Federal University of Ceará, Fortaleza, Brazil
Key words
p53 overexpression, refractory anemia
Correspondence
Sílvia M.M. Magalhães, Federal University of Ceará – Av.
José Bastos 3390 – CEP 60.436-160, Fortaleza, CE –
Brazil. Phone/Fax: international +55-85-2816221/+55-
85-2831854
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A complex immunodeficiency. Idiopathic CD4
+
T-lymphocytopenia and hypogammaglobulinemia
associated with HHV8 infection,
Kaposi’s sarcoma and gastric cancer
Sir,
We report a case of idiopathic CD4
+
T-lymphocy-
topenia (ICL) associated with hypogammaglobu-
linemia in a 70-year-old woman. She developed
Kaposi’s sarcoma (KS) and her mononuclear cells
were found to be positive for Herpes virus type 8
(HHV8). In 1997 she developed gastric cancer and
died fro m septic sho ck.
At the beginning o f 1990 unusual cases o f CD4
+
T-
lympho cyto penia in the absence o f human immuno -
deficiency virus (HIV) infection were reported.
1,2
In
1993 the CDC defined the criteria for a new syn-
drome: ICL. The criteria are lo w CD4
+
T-lymphocytes
(less than 300/ μL o r belo w 20% o f the to tal lympho -
cyte count), no serologic evidence of HIV infection
and no defined immuno deficiency diseases o r thera-
py asso ciated with T-cell depletio n.
3
In 1994 a 70-year-old woman with hypertension
and herpes zoster virus infection was admitted to our
sectio n because o f fever, skin abscesses due to Serra-
tia marcescens and angio mato us abscesses o n the left
leg. There was no epidemiology suggestive of HIV
Scientific correspondence
Figure 1. Nuclear staining for p53 (red) in the bone marrow
of a patient with MDS before (left, x200) and after pro-
gression (right, x400).
©Ferrata Storti Foundation