LETTER TO THE EDITOR
Pancytopenia and severe sepsis in an adult case of congenital
X-linked agammaglobulinemia (XLA)
Andrea Tendas
&
Pasquale Niscola
&
Teresa Dentamaro
&
Luca Cupelli
&
Gigliola Di Matteo
&
Andrea Finocchi
&
Agostina Siniscalchi
&
Stefano Fratoni
&
Teresa Scimò
&
Laura Scaramucci
&
Marco Giovannini
&
Micaela Ales
&
Alessio Pio Perrotti
&
Paolo de Fabritiis
Received: 9 May 2009 / Accepted: 21 December 2009 / Published online: 15 January 2010
#
Springer-Verlag 2010
Dear Editor,
Pancytopenia is a common cause of patient’s admission in a
hematology ward, usually due to neutropenia-related
infections, like pneumonia. Other than usually recorded
pathogenetic mechanisms, such as bone marrow (BM)
failure due to infiltrative malignant diseases or by the
several causes of BM aplasia, pancytopenia may be due to
rare causes, which should be carefully taken into account in
order to avoid mistaken diagnosis and to establish a correct
treatment approach, as demonstrated by the case recently
observed and thereby reported by us.
A 34-years-old man was admitted in the emergency
room of our hospital because of acute abdomen, which
occurred after some days of antibiotic-resistant fever
accompanied by a hemorrhagic and inflammatory lesion
involving the skin of the left foot. At admission, he was
pale and suffering; the physical examination revealed a
diffuse abdominal resistance. The past personal and familial
medical histories were not contributory. The initial ultra-
sound showed a gallbladder with an ultrasonographic
Murphy sign, a significant wall thickening over 5 mm and
pericholecystic fluid. The patient underwent abdominal
computed tomography (CT) scans, which confirmed posi-
tive findings for acute acalculous cholecystitis only. A
comprehensive laboratory evaluation revealed severe pan-
cytopenia with leucopenia (white blood cells=0.23×10
3
/
mcL with 0.020×10
3
/mcL neutrophil), mild anemia (he-
moglobin=10.9 g/dL), and thrombocytopenia (platelet
count=88×10
3
/mcL). A BM aspirates was then performed;
the evaluation of BM smears revealed a normal BM
cellularity with absence of megakaryocytic features and an
increase in the number of mitotic erythroid (30%) and
granulocytic (70%) cells; a maturative arrest of granulocy-
topoiesis with excess of promyelocytic (90% of granulo-
blasts), without blasts or hemophagocytosis, was observed
(Fig. 1). No lymphocytes were detected. A provisional
diagnosis of acute myeloid leukemia was made. Soon after,
the patient developed a sudden respiratory failure and a
severe shock with renal impairment, requiring admission in
intensive care unit where he received hemodynamic and
respiratory support, while an antimicrobic therapy with
meropenem and amikacin was started. A chest CT scan
revealed lung infiltrates suggesting a bilateral pneumonia,
associated with multiple bronchiectasia. Blood cultures
were negatives. Bronchoalveolar lavage was not performed
due to patient’s poor clinical conditions. The patient’s
clinical conditions slowly improved. A CT scan performed
1 week after the start of the antibiotic therapy revealed only
a mild reduction of lung infiltrates; cutaneous lesion of foot
A. Tendas
:
P. Niscola
:
T. Dentamaro
:
L. Cupelli
:
A. Siniscalchi
:
T. Scimò
:
L. Scaramucci
:
M. Giovannini
:
M. Ales
:
A. Pio Perrotti
:
P. de Fabritiis
Hematology Unit, Sant’Eugenio Hospital,
Rome, Italy
S. Fratoni
Pathology Department, Sant’Eugenio Hospital,
Rome, Italy
G. Di Matteo
:
A. Finocchi
Department of Public Health, Tor Vergata University,
Rome, Italy
A. Finocchi
Division of Immunology and Infectious Diseases,
Children’s Hospital Bambino Gesù,
Rome, Italy
A. Tendas (*)
Hematology Division, Sant’Eugenio Hospital,
Piazzale dell’Umanesimo 10,
00144 Rome, Italy
e-mail: tendas.andrea@aslrmc.it
Ann Hematol (2010) 89:949–951
DOI 10.1007/s00277-009-0891-7