12th Congress of the European Hematology ... - Haematologica

1217
INCIDENCE OF EARLY STAGE IDIOPATHIC MYELOFIBROSIS:
SINGLE INSTITUTION EXPERIENCE
G. Mele, P. Boccassini, M. Girasoli, C. Cristofalo, M.R. Coppi,
C.M. Brocca, A. Melpignano, G. Quarta
Antonio Perrino Hospital, BRINDISI, Italy
Background. Early stage idiopathic myelofibrosis with associated thrombocytosis
(i.e. prefibrotic myelofibrosis and early idiopathic myelofibrosis)
are with difficulty distinguished from true essential thrombocytemia
because of the high platelet count-sometimes greater than 1.000 × 109 /Las
well as the lack of specific diagnostic markers (i.e. evident
splenomegaly; high serum levels of LDH; circulating immature myeloid
cells, erythroblasts and dacryocytes). In these select circumstances, only
bone marrow features unequivocally help distinguish true essential
thrombocytemia from early stage idiopathic myelofibrosis with associated
thrombocytosis. In essential thrombocytemia megakaryocytes are
mostly giant and contain an enlarged nucleus with deep lobulations, but
lack the cytological abnormalities. In prefibrotic or early idiopathic
myelofibrosis megakaryocytes present bizarre forms with marked
nuclear-cytoplasmic anomalies. These evident dysplastic features exert a
significant impact on prognosis and life expectancy; in fact, life expectancy
is normal in true essential thrombocytemia but significantly shortened
in prefibrotic myelofibrosis as well as in the various fibrotic stages of
myelofibrosis. In addition, prefibrotic stages account for about 20-25%
of all idiopathic myelofibrosis. Methods and Results. We have evaluated the
incidence of early stages in our adult patients affected by idiopathic
myelofibrosis. The data of 32 patients (19 males, 13 females; median age
71 years [range 35-84]) were retrospectively analysed; 7 patients (22%)
were affected by early stage idiopathic myelofibrosis (4 prefibrotic
myelofibrosis, 3 early idiopathic myelofibrosis). Analysis was focused
on the discriminating impact of bone marrow and peripheral blood morphology.
Our patients with early stage idiopathic myelofibrosis showed
at diagnosis pronounced thrombocythemia (median value 879×109 /L
[range 427’1.654]). The number of WBC was not significantly elevated
(median value 13×109 /L [range 6-15]). Morphological examination of
peripheral blood did not show evidence of immature myeloid cells or
erythroblasts; the red blood cells were normocromic and normocytic in
all patients however minimal anisocytosis was observed during the
course of disease; in addition, peripheral blood smear showed frequently
macro-platelets, agranular platelets and small aggregates. The bone
marrow biopsies were hypercellular and dominated by atypical immature
megakaryocytes, conspicuously large due to an increase of nuclear and
cellular size, and always grouped in clusters of four/six elements; reticulin
fibrosis was minimal (MF1) or absent (MF0). At diagnosis and during
overall clinical follow-up all patients were asymptomatic: in fact they
did not present fatigue, pruritus, fever, weight loss, bone pain, night
sweats, spleen pain. Physical exam and abdominal echography detected
occasionally very small splenomegaly. Cytogenetic analysis revealed a
normal kariotype. LDH serum levels were normal. Conclusion. 1) In our
Institution the incidence of early stage of idiopathic myelofibrosis is similar
to that of international reports; 2) prefibrotic or early idiopathic
myelofibrosis are characterized by a relatively indolent clinical course; 3)
in view of the lack of specific diagnostic markers a detailed evaluation of
bone marrow findings, particularly megakaryopoiesis, is recommended
for a differential diagnosis between true essential thrombocytemia and
prefibrotic or early idiopathic myelofibrosis with associated thrombocytosis;
abnormal megakaryopoiesis offers the possibility of identifying early
stages idiopathic myelofibrosis apart from the platelet count, laboratory
parameters and clinical symptoms.
1218
HIGH DOSE SEQUENTIAL CHEMOTHERAPY AS SALVAGE TREATMENT FOR RELAPSED
AND REFRACTORY HODGKINS LYMPHOMA
L. Nassi, L. Rigacci, S. Guidi, L. Lombardini, C. Nozzoli, A. Gozzini,
S. Mappa, B. Puccini, V. Carrai, R. Alterini, R. Saccardi, A. Bosi
Careggi Hospital, FL, Italy
Background. The optimal approach for patients with Hodgkin’s lymphoma
(HD) who do not achieve a complete remission (CR) or relapse
after induction therapy is still not definite. High-dose chemotherapy with
autologous stem cell rescue proved to be more effective than conventional
schemes considering the number of second CR and the duration
of response. The German Hodgkin Study Group obtained good results
with high-dose sequential chemotherapy (HDST) and autologous stem
cell transplantation (ASCT) in this subset of patients. Aims. Evaluate the
12 th Congress of the European Hematology Association
impact of HDST in relapsed and refractory HD patients. Methods. Since
March 2002 to July 2006 16 patients were enrolled in this study in our
institution. The schedule was constituted by a first phase (2 cycles of
DHAP), a second phase (cyclophosphamide 4g/sqm, methotrexate 8
g/sqm, etoposide 2 g/sqm every 14 days) and an ASCT with BEAM conditioning.
All phases but the ASCT one were delivered on outpatient
basis. After the first phase a CT evaluation was performed to assess the
chemosensibility: patients not achieving at least a partial response (PR)
according to Cheson were considered off-study. Leukapheresis was performed
after high-dose cyclophosphamide, and G-CSF 5 microg/Kg was
administered from day +5 until collection. Results. Patients status at the
enrolment were: 7 relapsed within an year after the obtainment of CR;7
refractory to the induction therapy (ABVD); 2 refractory to conventional
salvage treatment for relapse occurred after 9 years. Characteristics of
the patients at diagnosis were: median age 28 years (range 19-42); 8
patients were female; 7 had B symptoms; ECOG performance status
were 0 in 10 patients, 1 in five, 2 in one. All patients received the two
cycles of DHAP without delays. After this phase 7 patients were excluded,
5 for stable/progressive disease, 2 for not achieving a PR (the late
relapsed patients, who are still alive with disease). Nine patients completed
the therapy according to the schedule, and after the ASCT all of them
were in CR with negative PET scans. A CR patient developed a myelodisplastic
syndrome 13 months after the ASCT and died from acute graft versus
host disease; a patient relapsed after 139 days, and died of progressive
disease. The other seven patients are still alive and maintain the CR.
Median time to treatment failure for the nine patients who completed the
HDST was 31 months (range 139 days-52 months). Conclusions. HDST is
an effective therapy for treatment of relapsed and refractory Hodgkin’s
lymphoma. We observed a better outcome in the relapsed patients than
in the primary refractory ones. After a median observation of more than
30 months we observed only a case of myelotoxicity, but a longer followup
could better evaluate the real toxicity of this treatment.
1219
INHIBITORS IN HEMOPHILIA A AND B IN SOUTEAST OF TURKEY
S. Pasa, A. Altintas, T. Cil, Y. Atayan, O. Ayyildiz
Dicle University Medical Faculty, DIYARBAKIR, Turkey
Background. The most important treatment related complication in
hemophiliacs is inhibitor development. We evaluate the inhibitor developing
rate of our patients, and discuss the possible reasons of low
inhibitor development rates of them. Materials and Methods. We performed
inhibitor screening of 99 hemophiliacs. Patients with < 1% of
clotting factor activities were classified as severe, 1-5% moderate, and
> 5-25% mild degree. Standart Bethesda procedure was performed for
inhibitor screening, and used of 0.6 units as cut-off level. Results. Out of
99 patients; 84 were hemophilia A (84.8%), and 15 were hemophilia B
(15.2%). Out of 84 hemophilia A patients; 53 (63.1%) were severe, 24
(28.5%) were moderate, and 7 (8.4%) were mild hemophilia A. Out of
15 hemophilia B patients; 8 (53.3%) were severe, 5 (33.4%) were moderate,
and 2 (13.3) were mild hemophilia B. Only one hemophilia A
patient was inhibitor positive (1.2% in severe hemophilia A), followed
in our service with severe intraabdominal bleeding. None of hemophilia
B patients have inhibitor. Conclusions. Expected inhibitor development
incidence in hemophilia A and B approaches 33% and 3% respectively.
Risk factors for inhibitory development may be patient and/or treatment-related.
Our patients inhibitor prevalence was lower than previous
studies. Factors that may responsible from this results; non of our
patients were administered prophylactic factor concentrates, using fresh
frozen plasma instead of factor concentrates. Inhibitor development
become an important problem in our region with increasing availability
of factor concentrates in prophylaxis and bleeding episodes.
1220
EFFECT OF PALIFERMIN ON THE INCIDENCE OF MUCOSITIS, HOSPITAL STAY, ACUTE
GVHD AND TOTAL PARENTERAL NUTRITION IN PATIENTS UNDERGOING STEM CELL
TRANSPLANTATION
N. D’Cunha, B. Williams, M. Chriva-Internati, E. Cobos
Texas Tech University, Health Sc Ctr, LUBBOCK, TEXAS, USA
Kepivance is a keratinocyte growth factor (KGF) which stimulates the
growth of cells in skin, mouth, stomach and colon. It has been suggested
that Kepivance may reduce the incidence and severity of oral mucositis
in patients receiving total body irradiation (TBI) conditioning regimen
followed by autologous stem cell rescue. Very few studies have shown
the effect of Kepivance on chemotherapy induced mucositis in patients
haematologica/the hematology journal | 2007; 92(s1) | 445