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

0710
CLINICO-PATHOLOGICAL FEATURES, RESPONSE TO FIRST-LINE THERAPY AND SURVIVAL
OF A LARGE SERIES OF PATIENTS WITH PRIMARY CUTANEOUS LYMPHOMA FROM AN
ACADEMIC REGIONAL HOSPITAL IN ITALY
G. Goteri, 1 S. Rupoli, 2 S. Pulini, 3 A. Tassetti,2 P. Picardi, 2 M. Ottaviani, 4
E. Grilli Cicilioni, 5 A.R. Scortechini, 2 S. Mulattieri, 2 A. Stronati, 2
A. Campanati, 6 A.M. Offidani, 6 S. Serresi, 7 L. Bugatti, 8 D. Brancorsini, 1
P. Leoni 2
1 Institute of Pathology, TORRETTE DI ANCONA; 2 Clinic of Hematology,
TORRETTE DI ANCONA; 3 Department of Hematology, Pescara Hospit,
PESCARA; 4 Division of Dermatology, FABRIANO; 5 Division of Dermatology,
S.Severino Hosp, SAN SEVERINO; 6 Clinic of Dermatology, ANCONA; 7 Division
of Dermatology, INRCA, Ancona, ANCONA; 8 Division of Dermatology,
Jesi Hospital, JESI, Italy
Background/Aims. 2005-WHO/EORTC classification for primary cutaneous
lymphomas (PCL) has provided clear-cut diagnostic criteria, relative
frequency and patients’ survival for each entity. We tested the utility
of this classification and calculated relative frequency and survival for
each entity in our series of PCL patients. Methods. Of all our PCL patients
diagnosed and treated from 1990 to 2006, diagnoses were reviewed
according to the WHO/EORTC scheme and the clinical data retrieved.
Overall and major/minor-event-free survival (OS, major/minor-EFS) were
calculated by Kaplan-Meier method. Results. Clinico-pathological features:
Our patients were 259, 170 M/89 F (median age, 62 yrs): 191
Mycosis Fungoides (MF: 185 classic/6 folliculotropic type; 168 stage IA-
B, 14 IIA-B, 6 IIIA-B, 3 IVA); 43 cutaneous B-cell lymphomas (CBCL: 25
follicular; 14 marginal zone; 3 diffuse large B-cell lymphoma-leg type; 1
lymphoblastic lymphoma); 10 Sézary Syndrome (SS); 7 CD30 + lymphoproliferative
disorders (7 Lymphomatoid Papulosis-LyP); 8 rare cutaneous
T-cell lymphomas (rare-CTCL: 5 peripheral T-cell lymphoma
unspecified, 1 g/d lymphoma, 1 CD4 + /CD56 + Hematodermic Neoplasia,
1 T-cell lymphoblastic lymphoma; all stage IV). First lesions appeared at
a mean age of 54 yrs, with a mean interval to the diagnosis of 57 mo.s.
Patients had patches in 57%, nodules in 20%, plaques/papules in 17%
and erythroderma in 6%. Lesions were disseminated in 57%, multiple
with regional distribution in 30% and single in 13%. Pruritus was the
most frequent symptom (46%). First-line therapies consisted of phototherapy
and interferon in 54%; mono-/poli-chemotherapy in 14%;
heliotherapy in 13%; radiotherapy in 8%, surgery in 6%; other therapies
in 3%; no therapy in 2%. Response to therapy: The overall response
rate was 100% in LyP (all complete remissions-CR), 93% in CBCL (88%
CR), 92% in MF (81% CR), 50% in rare-CTCL (38% CR) and in SS
patients (20% CR). Median time to CR was 1 month in CBCL, 2 mo.s
in rare-CTCLs, 5 in MF, 7 in SS and 13 in LyP. Follow-up: 30% MF, 16%
CBCL and 14% LyP patients experienced a minor event. Median minor-
EFS rate was 71 mo.s. A major event occurred in 75% rare-CTCL, 30%
SS, 14% CBCLs and 7% MF patients. Major-EFS was significantly worse
in rare-CTCL patients (median 6 mo.s). In MF the major-EFS was statistically
significantly worse in patients with tumors/erythroderma (median,
18/19 mo.s) than in patients with patches (98% at 186 mo.s) and
papules/plaques (80% at 83 mo.s). At last follow-up 35 patients (13%)
were dead. Median OS was 156 mo.s, significantly influenced by the
lymphoma type: 10 mo.s in rare-CTCLs, 135 in SS and 115 in CBCLs;
patients with LyP (100% rate at 180 mo.s) and MF (83% rate at 140
mo.s) behaved much better. Summary and Conlusions. Our series confirms
the clinical utility of the WHO/EORTC classification which segregates
patients with different outcome. MF at initial stage and CBCLs
are indolent disorders with a risk of minor events, whereas advanced MF,
SS and particularly rare-CTCLs behave aggressively. PCL subtype relative
frequences in our series differs from WHO/EORTC data maybe due
to a peculiar case-selection in a Hematological setting.
0711
PATHOLOGY AND CLINICAL COURSE OF MALT LYMPHOMA WITH PLASMACYTIC
DIFFERENTIATION
S.W. Wohrer, M. Troch, B. Streubel, L. Muellauer, A. Chott,
M. Raderer
Medical University of Vienna, VIENNA, Austria
Background. The feature of plasmacytic differentiation (PD) is present
in up to 30% of patients diagnosed with MALT lymphoma. To date, the
influence of PD on the clinical course of MALT lymphoma has not been
assessed. Aims and Methods. Therefore, we have retrospectively analysed
the clinical characteristics and the course of the disease in 34 (25%)
12 th Congress of the European Hematology Association
patients with PD as compared to 101 (75%) MALT lymphoma-patients
without this histological feature. Results. Patients with PD had significantly
more extragastic lymphomas (28/34 (83%) vs. 54/101 (53%), p=0.003)
and a significantly lower rate of t(11;18) (2/26 (8%) vs. 22/72 (31%),
p=0.02). There was no significant difference of age at diagnosis (62 years
vs. 64 years, p=0.64), relapse rate (48% vs. 37%, p=0.27), median time
to relapse (26.5 months vs. 29 months, p=0.84), monoclonal gammopathy
(50% vs. 44%, p=0.63), t(14;18) involving IGH/MALT1 (11% vs.
8%, p=0.68), trismomy 3 (31% vs. 27%, p=0.69), trismomy 8 (8% vs.
10%, p=0.74) and the presence of autoimmune diseases between both
groups (53% vs. 37%, p=0.09). Conclusion. In conclusion, we found that
PD is predominantly found in extragastric MALT lymphoma but has no
significant impact on clinical course and prognosis.
0712
A VALIDITY STUDY OF CYTOMORPHOLOGY AND FLOW CYTOMETRY IN LYMPH NODE
BIOPSIES FROM PATIENTS WITH ADENOPATHIES
M. Colorado, 1 M. Cuadrado, 1 F. Mazorra, 1 O. Acinas, 2 A. Bermudez, 1
A. Insunza, 1 L. Yañez, 1 A. Iriondo1 1 2 Hospital Valdecilla, SANTANDER; Hospital de Sierrallana, TORRELAVE-
GA, Spain
Background. Cytomorphology and flow cytometry may be an useful
diagnostic test in tissues from patients with adenopathies. Objective. The
aim of this study was to assess the efficacy of flow cytometry for diagnosis
of lymphoma in patients with lymphadenopathies with or without
suspected haematological disorders. Material and Methods. The study
was conducted from January 2004 through February 2007. The study
was designed with 90% power at a significance level of 0.05 to detect a
sensitivity of 70% or more, assuming an overall incidence of lymphoma
of 38% (EPIDAT 3.1 program).We prospectively evaluated the cytomorphology
and the immunophenotype of 268 consecutive biopsies. Cytomorphology
of touch imprints (May Grünwald Giemsa) and flow
cytometry were performed on fresh material. All biopsies underwent histologic
and immunohistochemistry study (Gold Standard Study). The following
combinations of monoclonal antibodies were systematically used
in the immunophenotype study: CD3/CD4/CD8/CD45, CD19/
CD5/CD20/CD45, CD19/CD10/CD20/CD45, CD19/sIgK/sIgL. In certain
cases, the study was expanded with other B, T, NK or myeloid monoclonal
antibodies in accordance with the initial immunophenotype
Results. Cytomorphology and flow cytometry studies and histologic and
immunohistochemistry studies were interpreted independently by
hematologists and pathologists respectively. The samples were categorized
positive if an aberrant phenotype was detected by flow cytometry
or if Hodgkin cells were detected in the touch imprint. Results. Both
studies were completed in 239 biopsies. 29/268 (10.8%) samples were
not analyzed using flow cytometry because of necrosis or insufficient
number of cells. The biopsies were performed in 142 males (53%) and
126 females (48%). The mean age was 51 years (1-93). In 24/239 (10%)
cases, there was a previous history of lymphoma. The most common
locations of lymphadenopathies were abdominal 41 (15%), inguinal 32
(12%), otolaryngologic 30 (11%), supraclavicular 26 (9%), axillary 18
(7%), mediastinal 13 (5%), splenic 2 (0.7%), and other locations 10 (4%).
The cytomorphology and flow cytometry were significantly faster in
recognizing lymphomas than classical pathological examination (1.8
days versus 8.7 days respectively p