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Sezary syndrome (SS) treated successfully with fludarabyne: Case report
Posters - Urticaria and mast cell disorders PUVA at gradually reduced doses even two months. After the induction phase at least a partial remission was achieved in all patients. In the maintenance phase 1 to 5 months after, a complete remission (CR) of cutaneous lesions was observed in 16/21 patients (76%) and a PR was seen in 3/2l patients (140/o), thus accounting for an overall response rate of 90%. Only one patient suffering from previous acute myeloid leukemia was unresponsive and one patient was excluded from the protocol for intolerance (cutaneous rash). During the follow-up period 3 patients developed local recurrences; repetition of the initial treatment schedule again led to a CR in 2/3 cases and to a PR in 113 cases. Furthermore, in a median period of 18 months from the maximum response achieved up to the last evaluation, 15/21 patients (71%) are still in CR and 4/2l patients (19%) present stable PR. Evaluation of toxicity was carefully monitored during the clinical trial. Mild gastrointestinal side effects (grade I) and flu-like syndrome were observed in 7 and 4 patients, respectively; nausea and vomiting (grade II) were seen in one patient; one patient presented mild leukopenia (grade I), another patient reversible depression and one patient showed cutaneous toxicity (grade II). The results we obtained (7 1% CR, 19% PR) are similar to those reported by other combination studies. ElP543 Sezary syndrome (SS) treated successfully
with fludarabyne: Case report
Cl. Brandozzi’, I. Cataldi’, S. Rupol?, S. Barulli3, B. Guiducci3, M. Giangiacomi4, P. Leoni3. ‘Diijisione di Dermafologia; ’ Clinica di Dermatologia; 3Clinica di Ematologia; ‘Auafomia Patologica, Vniversitd di Ancona, Italy A patient, of sixty-eight years of age, has been hospitalized for erythrodermia, characterized by slight fever, loss of weight, itching and poliadenopathy. The diagnosis expressed had been of SS. The patient was treated at first, with local therapy without success, and, afterwards, with IFN 2b at l-8 MU dose a week. This therapy was precociously interrupted because of the onset of heart toxicity. A course of therapy with MTX 5-10 mg/ms dose a week per OSdetermined a remarkable outcome on the clinical and objective level but also important phenomena of toxicity. Afterwards, a monotherapy was started, represented by the infusion of fludarabyne 25 mg/m’/die for five consecutive days/a month for 4 courses together with low doses of steroid. The treatment induced the disappearance of itching and the complete resolution of skin lesions both an the clinical and histological level and the drastic resolution of the levels of moving lymphocytes CD4+. As everybody knows from the literature, the problem of the treatment of SS is unresolved. No therapy is able to induce a rate of responses exceeding 50%. Recently encouraging results have been obtained with low doses of MTX. In the case described the Fludarabyne used as the only agent has induced a complete histological persistent remission after a year of observation and the complete disappearance of the lymphocytosis CD4+. However, further studies are necessary to confirm the use of Fludambyne in the treatment of patients who suffer of SS.
IP544
s313
Leukemia cutis in acute leukemlas: A review of 186 patients
G. Brandozzi’, S. Rupol?, L. Ombrosi2, L. Corvatta’, S. Barulli2. B. Guiducci’, M. OfIidani*, M. Giangiacomi3, P. Leoni*. ‘Divisione di Demmtologia; 2Clinica di Ematologia; jAnaromia Patologica Vniversitd di Ancona, Italy The cutaneous manifestations of leukemias can be divided into non specific lesions and lesions containing leukemic cells (leukemia cutis). A wide variety of non specific cutaneous eruptions including drug reactions, vasculitis, lesions secondary to infections or bleeding diathesis are seen in leukemic patients. Leukemia skin infiltrates are uncommon and include a broad category of myeloproliferative disorders in their acute and chronic form. Skin biopsies were classified as “concomitant” if the diagnosis of systemic leukemia was established as the same time as the skin biopsy. If the hemathologic diagnosis was established prior to the skin biopsy, these cases are classified as “pre-dating”; furthermore Leukemia cutis (LC) rarely represents an isolate site of relapse. We observed 186 patients: 136 were affected by Acute Non Lymphoblastic Leukemia (ANLL) and 50 by Acute Lymphoblastic Leukemia (ALL), 119 males and 67 females, median age 51.5 years (range 13-89 years). We demonstrated a 3.8% incidence of LC (7 patients) on the whole. In 3 cases (I .6%) cutaneous foci of leukemia were noted at diagnosis of acute leukemias, in 4 cases (2.7%) LC occurred after the diagnosis of acute leukemias, while 2 cases (1.1%) represented isolated sites of relapse, which occurred during bone marrow remissions. Clinically skin lesions typicahy manifested as papules or subcutaneous nodules. A particular high incidence of LC was noted in certain sub-groups of patients with higher predominance of monoblastic varietes of ANLL (5 M4, lM5b). In litemture LC has been associated to a peculiar expression of blast cell surface markers. Our immunophenotypic data confirmed that the presence of LC is associated to the absence of CD14 monocytic blasts, furthermore monocytic blast expression of T cell antigens (CD2, CD7) was seen in 2 patients. The cell type of skin infiltrates detected histologically and by a panel of antibodies and enzymes, always reflected the type of leukemias diagnosed from bone marrow and peripheral smears. Our report confirms that the presence of cutaneous leukemic lesions is associated with a poor outcome: 5 patients died after 35 days on average (range 15-110 days) from the appearance of the skin lesions.
Urticaria and mast cell disorders Mastocytosis skin mast cells morphologlcal characteristics based on computer analysis system A. Zalewska’, M. Strzelecki*, P. Janowski’, A. Omulecki’. ‘Department of Dermatology, Medical Academy, L&i.& 2Technical University of f.ddz Poland Mastocytosis refers to a fairly large spectrum of conditions characterized by mast cell hyperplasia. Mast cells can .be visualized using specific staining procedures. Pinacyanol erythrosinate belongs to very sensitive stains for mast cells. .Due
with fludarabyne: Case report
Cl. Brandozzi’, I. Cataldi’, S. Rupol?, S. Barulli3, B. Guiducci3, M. Giangiacomi4, P. Leoni3. ‘Diijisione di Dermafologia; ’ Clinica di Dermatologia; 3Clinica di Ematologia; ‘Auafomia Patologica, Vniversitd di Ancona, Italy A patient, of sixty-eight years of age, has been hospitalized for erythrodermia, characterized by slight fever, loss of weight, itching and poliadenopathy. The diagnosis expressed had been of SS. The patient was treated at first, with local therapy without success, and, afterwards, with IFN 2b at l-8 MU dose a week. This therapy was precociously interrupted because of the onset of heart toxicity. A course of therapy with MTX 5-10 mg/ms dose a week per OSdetermined a remarkable outcome on the clinical and objective level but also important phenomena of toxicity. Afterwards, a monotherapy was started, represented by the infusion of fludarabyne 25 mg/m’/die for five consecutive days/a month for 4 courses together with low doses of steroid. The treatment induced the disappearance of itching and the complete resolution of skin lesions both an the clinical and histological level and the drastic resolution of the levels of moving lymphocytes CD4+. As everybody knows from the literature, the problem of the treatment of SS is unresolved. No therapy is able to induce a rate of responses exceeding 50%. Recently encouraging results have been obtained with low doses of MTX. In the case described the Fludarabyne used as the only agent has induced a complete histological persistent remission after a year of observation and the complete disappearance of the lymphocytosis CD4+. However, further studies are necessary to confirm the use of Fludambyne in the treatment of patients who suffer of SS.
IP544
s313
Leukemia cutis in acute leukemlas: A review of 186 patients
G. Brandozzi’, S. Rupol?, L. Ombrosi2, L. Corvatta’, S. Barulli2. B. Guiducci’, M. OfIidani*, M. Giangiacomi3, P. Leoni*. ‘Divisione di Demmtologia; 2Clinica di Ematologia; jAnaromia Patologica Vniversitd di Ancona, Italy The cutaneous manifestations of leukemias can be divided into non specific lesions and lesions containing leukemic cells (leukemia cutis). A wide variety of non specific cutaneous eruptions including drug reactions, vasculitis, lesions secondary to infections or bleeding diathesis are seen in leukemic patients. Leukemia skin infiltrates are uncommon and include a broad category of myeloproliferative disorders in their acute and chronic form. Skin biopsies were classified as “concomitant” if the diagnosis of systemic leukemia was established as the same time as the skin biopsy. If the hemathologic diagnosis was established prior to the skin biopsy, these cases are classified as “pre-dating”; furthermore Leukemia cutis (LC) rarely represents an isolate site of relapse. We observed 186 patients: 136 were affected by Acute Non Lymphoblastic Leukemia (ANLL) and 50 by Acute Lymphoblastic Leukemia (ALL), 119 males and 67 females, median age 51.5 years (range 13-89 years). We demonstrated a 3.8% incidence of LC (7 patients) on the whole. In 3 cases (I .6%) cutaneous foci of leukemia were noted at diagnosis of acute leukemias, in 4 cases (2.7%) LC occurred after the diagnosis of acute leukemias, while 2 cases (1.1%) represented isolated sites of relapse, which occurred during bone marrow remissions. Clinically skin lesions typicahy manifested as papules or subcutaneous nodules. A particular high incidence of LC was noted in certain sub-groups of patients with higher predominance of monoblastic varietes of ANLL (5 M4, lM5b). In litemture LC has been associated to a peculiar expression of blast cell surface markers. Our immunophenotypic data confirmed that the presence of LC is associated to the absence of CD14 monocytic blasts, furthermore monocytic blast expression of T cell antigens (CD2, CD7) was seen in 2 patients. The cell type of skin infiltrates detected histologically and by a panel of antibodies and enzymes, always reflected the type of leukemias diagnosed from bone marrow and peripheral smears. Our report confirms that the presence of cutaneous leukemic lesions is associated with a poor outcome: 5 patients died after 35 days on average (range 15-110 days) from the appearance of the skin lesions.
Urticaria and mast cell disorders Mastocytosis skin mast cells morphologlcal characteristics based on computer analysis system A. Zalewska’, M. Strzelecki*, P. Janowski’, A. Omulecki’. ‘Department of Dermatology, Medical Academy, L&i.& 2Technical University of f.ddz Poland Mastocytosis refers to a fairly large spectrum of conditions characterized by mast cell hyperplasia. Mast cells can .be visualized using specific staining procedures. Pinacyanol erythrosinate belongs to very sensitive stains for mast cells. .Due