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G Avvisati

Publications and source records attributed to G Avvisati.

At least 55 records · Page 3Linked to original sources

Etoposide, intermediate-dose cytarabine and carboplatin (VAC): a combination therapy for the blastic phase of chronic myelogenous leukemia.

BACKGROUND: Carboplatin is a second-generation platinum compound that in combination with etoposide and intermediate doses of cytarabine, in early clinical trials has demonstrated high efficacy in refractory acute myelogenous leukemia and the blastic phase of CML. PATIENTS AND METHODS: Starting in April 1992, 17 consecutive patients with the blastic phase (BP) of chronic myelogenous leukemia (CML) and a median age of 33 years (range 10 to 45) received a new treatment regimen consisting of etoposide (100 mg/m2/d i.v. over one hour), intermediate-dose cytarabine (500 mg/m2/d i.v. over one hour, q12 hours) and carboplatin (150 mg/m2/d continuous infusion) on days 1 3 and 8 10 (VAC regimen). The BP phenotype was myeloid in 16 patients and hybrid in one. At the time of their BP diagnoses, two patients showed extramedullary involvement, in eight patients the BP was preceded by an accelerated phase. RESULTS: Overall, 11 of 17 patients (65%) achieved complete remission and 7 of 11 responding patients (64%) manifested a cytogenetic conversion ranging from 38% to 100% Ph-negative metaphases: one patient died in CR of hemothorax, three died of sepsis during induction therapy and three patients (17.5%) had resistant disease. As of April 1996, four patients are alive, three of them in first remission at +7, +10, +16 months after CR, and one in BP at +38 months after the start of VAC therapy. Profound myelosuppression was observed in all patients; extrahematologic toxicity, especially involved the gastro-intestinal tract, with grade > 2 mucositis in five patients (29.5%) and liver dysfunction in five (29.5%). No renal toxicity or ototoxicity was observed. CONCLUSIONS: Despite the brief relapse-free duration, the high remission rate and tolerable toxicity indicate that the VAC combination chemotherapy has a high antileukemic efficacy, and warrants further evaluation for the treatment of CML in acute phase, provided a post-remission BMT strategy is feasible.

Adolescent↗

The role of all-trans-retinoic acid (ATRA) treatment in newly-diagnosed acute promyelocytic leukemia patients aged > 60 years.

BACKGROUND: To evaluate the role and toxicity of ATRA therapy in newly-diagnosed APL patients aged > 60 yrs, the outcome of 16 consecutive elderly APL patients observed between January 1990 and June 1996 were analyzed. PATIENTS AND METHODS: Their median age was 65.5 yrs (range 60-81 years), the male/female ratio was 7:9, and molecular biology analysis showed a PML/RARa rearrangement in all patients. Seven patients had a concomitant cardiovascular disease. ATRA 45 mg/sqm/day was given to all patients, and in 11 was associated with idarubicin (AIDA protocol); in two patients ATRA was associated with mitoxantrone + ara-C, while the remaining three patients received ATRA alone. RESULTS: Fourteen patients (87.5%) achieved CR, and two patients (12.5%) died during induction. Despite the high CR rate, eight episodes of severe cardiovascular complication were observed in seven patients, three of whom had previously had cardiovascular disease; in addition, three patients had sepsis (two bacterial and one fungal). As of 31 March 1997, 9 of 14 patients were still in first CR after a 19-month (range 7-64 months) median follow-up since attainment of the CR. One patient died in CR of a fungal complication and four patients relapsed after 8, 9, 23 and 35 months following CR: two of them achieved a second CR lasting seven and +15 months with ATRA alone. Of the nine patients still in first CR, only three have received the planned consolidation therapy and five have been in CR for more than 24 months (+25, +33, +34, +38, +63). CONCLUSIONS: Despite the fact that most of these patients received shorter consolidation treatments than do younger patients, the good results achieved in them might be considered an indication for modifying treatment schedules in order to reduce severe toxicity and improve protocol compliance.

Aged↗

Conventional induction treatments do not influence overall survival in multiple myeloma.

A retrospective analysis was performed on two subsequent myeloma patient series treated with the same conventional induction treatments, melphalan and prednisone or alternating VMCP/VBAP: 273 were enrolled in the multicentre M83 trial (M83 trial group) from 1983 to 1986; 160 were referred to a single institution (Haemat. To group) from 1986 to 1994. Response to treatment was very similar in the two groups (53% v 50.3%). Remission duration curves merely overlapped (median 20 v 21 months). However, overall survival was significantly longer in the Haemat. To group (43.2 v 33 months, P < 0.04). This difference was due to a prolonged period from relapse or progression to death (21 v 8 months, P < 0.01; 20.8 v 7 months, P < 0.009). Prolonged survival was also observed in poor-prognosis patients with a serum beta2-microglobulin level > 3 mg/l, in the Haemat. To group (31.8 v 24.2 months, P < 0.04). The same induction treatments produced almost identical response rate and remission duration in both groups, but overall survival was 10 months longer for one group. However, it could be argued that treatment salvage modalities and support therapies have been improved in a decade. Lastly, induction treatments did not influence overall survival.

Adult↗

Current guidelines for the management of aggressive non-Hodgkin's lymphoma.

The prognosis of aggressive non-Hodgkin's lymphoma (NHL) has improved greatly during recent years with the use of combination chemotherapy. Planning the treatment must take into consideration the patient's age, performance status, histological subtype and disease extent and severity. Recently, a 4-part International Prognostic Index (IPI), based on 5 prognostic factors, has permitted the allocation of patients with NHL in 2 well defined prognostic groups: good prognosis (low and low-intermediate risk) and poor prognosis (intermediate-high and high risk). Conventional chemotherapy with CHOP (a chemotherapeutic regimen consisting of a combination of cyclophosphamide, doxorubicin, vincristine and prednisone) or other equivalent third-generation regimens may be considered the standard treatment for the good prognosis group. In the poor prognosis group the probability of long term survival is less than 40% with conventional chemotherapy. Therefore, an early intensification with high dose therapy following peripheral stem cell transplantation (PSCT) should be considered in the setting of randomised trials. Localised stage disease, defined as stages I-IE and II-IIE without adverse prognostic factors, has a very good prognosis with a long term survival exceeding 80% using brief conventional chemotherapy regimens plus involved field radiotherapy. Refractory or relapsing patients after the drugs of first choice are given who subsequently respond to salvage chemotherapy should be enrolled for a course of high dose consolidation chemotherapy followed by PSCT. Elderly patients without severe organ dysfunction can take advantage from specifically devised chemotherapy regimens, with a response rate similar to that of younger patients. However, despite major advances in the treatment of aggressive NHL, additional clinical trials are required to enable the clinician to define the best therapeutic programmes to treat patients with this disorder.

Aged↗

AIDA (all-trans retinoic acid + idarubicin) in newly diagnosed acute promyelocytic leukemia: a Gruppo Italiano Malattie Ematologiche Maligne dell'Adulto (GIMEMA) pilot study.

From March 1993 to October 1993, 20 consecutive, newly diagnosed acute promyelocytic leukemia (APL) patients from 13 Italian institutions entered in a pilot study named AIDA, combining all-trans retinoic acid (ATRA) with idarubicin (IDA). ATRA was administered orally beginning on the first day of induction at the dosage of 45 mg/m2/d until complete remission (CR), whereas IDA was administered intravenously at the dosage of 12 mg/m2/d on days 2, 4, 6, and 8 of the induction. Patients who achieved CR were consolidated with 3 courses of chemotherapy without ATRA; thereafter, they were followed up for molecular and hematologic CR. The median age was 35.3 years (range, 6.5 to 67.6 years); 8 patients were males and 12 females; 4 had the hypogranular variant of APL (M3v), and 4 (2 with M3v) presented with leukocyte counts > or = 10,000/microL. Molecular analysis for the promyelocytic leukemia-retinoic acid receptor alpha (PML-RAR alpha) hybrid gene at diagnosis was performed in 16 patients by means of reverse transcription-polymerase chain reaction (RT-PCR) analysis, and all were RT-PCR+ for the hybrid gene. In the remaining 4 patients, the cytogenetic study showed the presence of the t(15;17). After a median time of 36 days (range, 28 to 52 days) 18 (90%) patients achieved CR; the remaining 2 patients died 12 and 34 days after diagnosis from myocardial infarction caused by fungal myocarditis and from massive hemoptysis, respectively. ATRA syndrome was observed in only 2 patients, and, after the prompt discontinuation of ATRA and initiation of dexamethasone, both recovered from the syndrome. However, after recovering, 1 patient achieved CR, whereas the other died at day 34 because of massive hemoptysis; other side effects were very limited. At recovery from the third consolidation course, only 3 of 14 (21.4%) tested patients were RT-PCR+ for the PML-RAR alpha hybrid gene. Of these, 2 relapsed shortly afterwards; however, in the last patient, the PML-RAR alpha disappeared at successive testing performed 2 months later. As of September 30, 1995, after a median follow-up period from diagnosis of 27 months (range, 24 to 31 months), the overall survival and event-free survival durations are 85% and 69%, respectively; moreover, 14 of 18 (78%) patients who achieved CR are still alive and in first molecular and hematologic CR. Of the 4 relapsed patients, 3 achieved a second CR with ATRA and, after further treatment, are now in molecular and hematologic CR after 4+, 16+, and 17+ months from the second CR. These results indicate that (1) the AIDA protocol is highly effective in treating APL; (2) after 3 consolidation courses, the majority of patients who achieved CR are RT-PCR- for the hybrid gene PML-RAR alpha; (3) the persistence of an RT-PCR positivity for the PML-RAR alpha hybrid gene after 3 consolidation courses is indicative of early relapse, thus these patients still require additional treatment. These results have prompted the Gruppo Italiano Malattie Ematologiche Maligne dell'Adulto (GIMEMA) to initiate, in cooperation with the Associazione Italiana di Ematologia ed Oncologia Pediatrica and some European Organization for Research and Treatment of Cancer (EORTC) centers, a new multicentric clinical trial named AIDA LAP 0493 for the treatment of adult and pediatric APL patients. All patients are considered eligible if APL diagnosis is confirmed with molecular or cytogenetic studies for PML-RAR alpha hybrid gene or t(15;17) and are enrolled to receive the same induction and consolidation therapy of this pilot study. After consolidation, patients who are RT-PCR- for PML-RAR alpha hybrid gene are randomized to four arms, whereas patients who are RT-PCR+ after consolidation undergo, if eligible, an allogenic transplantation procedure.

Adult↗

Retinoid-induced differentiation of acute promyelocytic leukemia involves PML-RARalpha-mediated increase of type II transglutaminase.

All-trans retinoic acid (t-RA) administration leads to complete remission in acute promyelocytic leukemia (APL) patients by inducing growth arrest and differentiation of the leukemic clone. In the present study, we show that t-RA treatment dramatically induced type II transglutaminase (type II TGase) expression in cells carrying the t(15;17) translocation and expressing the PML-RARalpha product such as the APL-derived NB4 cell line and fresh leukemic cells from APL patients. This induction correlated with t-RA-induced growth arrest, granulocytic differentiation, and upregulation of the leukocyte adherence receptor beta subunit (CD18) gene expression. The increase in type II TGase was not abolished by cycloheximide treatment, suggesting that synthesis of a protein intermediate was not required for the induction. t-RA did not significantly alter the rate of growth arrest and did not stimulate differentiation and type II TGase activity in NB4.306 cells, a t-RA-resistant subclone of the NB4 cell line, or in leukemic cells derived from two patients morphologically defined as APL but lacking the t(15;17). However, in NB4.306 cells, t-RA treatment was able to increase CD18 mRNA expression in a manner similar to NB4 cells. The molecular mechanisms involved in the induction of these genes were investigated. In NB4 cells, using novel receptor-selective ligands such as 9-cis-RA, TTNPB, AM580, and SR11217, we found that RAR- and RARalpha-selective retinoids were able to induce growth arrest, granulocytic differentiation, and type II TGase, whereas the RXR-selective retinoid SR11217 was inactive. Moreover, an RAR alpha-antagonist completely inhibited the expression of type II TGase and CD18 induced by these selective retinoids in NB4 cells. In NB4.306 cells, an RARalpha-dependent signaling pathway was found involved in the modulation of CD18 expression. In addition, expression of the PML-RARalpha gene in myeloid U937 precursor cells resulted in the ability of these cells to induce type II TGase in response to t-RA. On the basis of these results we hypothesize a specific involvement of a signaling pathway involving PML-RAR alpha for the induction of growth arrest, granulocytic differentiation, and type II TGase by retinoids in APL cells.

Apoptosis↗

Treatment of high-risk myelodysplastic syndromes with lymphoblastoid alpha interferon.

UNLABELLED: Seventeen patients with high-risk myelodysplastic syndromes (HR-MDS) received lymphoblastoid-interferon alpha (Ly-IFN alpha) for 3 months at escalating doses from 0.5 to 3 MU s.c. 3 times per week. Three patients stopped the treatment after 2 months because of cardiac failure (one patient) and cerebral haemorrhage (two patients); six had a partial response (PR) and continued Ly-IFN alpha; six were resistant and stopped Ly-IFN alpha; two evolved to acute myelogenous leukaemia (AML). Among the six partial responders, four achieved a complete response (CR) during subsequent Ly-IFN alpha treatment (CR duration 3, 4+, 15+ and 29 months) and two did not achieve any further improvement (PR duration 3 and 9 months). Two resistant patients had an unexpected clinical improvement soon after Ly-IFN alpha discontinuation and achieved a PR of 6+ and 14+ months respectively. No toxicity related to Ly-IFN alpha treatment was observed and no reduction of dosage was needed. IN CONCLUSION: (1) Ly-IFN alpha seems to be effective in some patients with HR-MDS; (2) the best treatment duration seems to be of a least 6 months.

Adult↗

Meningeal localization in a patient with Hodgkin's disease. Description of a case and review of the literature.

We report the case of a 21-year-old man in whom intracranial localization was discovered during initial staging at the onset of Hodgkin's disease (HD). The patient was treated by surgical excision, irradiation and chemotherapy and 50 months after completion of therapy is in remission with no evidence of HD. A brief review of the literature regarding 48 patients with intracranial Hodgkin's disease is presented.

Adult↗

Simultaneous occurrence of large B-cell non-Hodgkin lymphoma and myelodysplastic syndrome rapidly evolving into acute myeloblastic leukemia.

We describe a case of simultaneous occurrence of large B-cell non-Hodgkin lymphoma and myelodysplastic syndrome in the absence of previous chemotherapy or radiotherapy. After initiation of steroid treatment, the myeloid clone showed a rapid increase in both the bone marrow and peripheral blood with transformation into acute myeloid leukemia. The diagnosis were confirmed by immunophenotypic studies performed in the histologic sections of the lymph node, as well as in bone marrow and peripheral blast cells. This case may be indicative of potential down-regulation of a malignant myeloid clone induced by the malignant lymphoid clone.

Aged↗

Improved rapid detection of the PML/RARalpha fusion gene in acute promyelocytic leukemia.

Acute promyelocytic leukemia (APL) is a medical emergency which requires rapid diagnosis and tailored treatment. Detection of the PML/RARalpha fusion gene in APL blasts is critical to start promptly the specific therapy with all-trans retinoic acid (ATRA). APL lacking this genetic lesion have been reported as being ATRA resistant. Reverse-transcription polymerase chain reaction (RT-PCR) has been extensively used to detect the PML/RARalpha cDNA. The reported PML/RARalpha amplification techniques are laborious and time consuming, and include conventional RNA extraction, cDNA synthesis and a two-round (nested) PCR. We hereby describe a few variations of the commonly adopted RNA extraction and PML/RARalpha RT-PCR protocols which allow a molecular diagnosis of APL to be carried out in less than 5 h. Processing of small volumes of leukemic cell lysate (0.5 ml) in a microfuge allows extraction of good quality RNA in 1 h. After reverse transcription to obtain cDNA, a 'hot start' PCR procedure was adopted which enabled us to amplify clearly visible and specific products after a single (not nested) amplification round. The PML/RARalpha fusion gene was detected in the blasts of six consecutive APL at diagnosis, and an APL-tailored protocol including ATRA was started in each case within 6 h of admission. On repeated experiments, the assay proved highly specific and sensitive for the rapid detection of all PML/RARalpha transcript types. Our data should encourage the use of this rapid procedure for the diagnosis of both typical APL and, particularly, less typical cases awaiting urgent therapeutic intervention.

Base Sequence↗

Outcome of patients with acute myeloid leukemia who failed to respond to a single course of first-line induction therapy: a GIMEMA study of 218 unselected consecutive patients. Gruppo Italiano Malattie Ematologiche Maligne dell'Adulto.

The outcome of a cohort of 218 consecutive patients who failed to respond to a single course of standard daunorubicin plus ARAC (three + seven) induction regimen has been retrospectively evaluated to assess the characteristics of this group of AML patients and the effectiveness of second-line induction programs. Seventy-four of the 218 patients (33.9%) attained complete remission with salvage chemotherapies. The multivariate analysis of pretherapy characteristics of the patients showed that peroxidase positivity and age were the most important factors in determining whether or not the patient would have a favorable response to second-line induction regimen. In addition, comparison of marrow characteristics at diagnosis with those of marrow after the first-line therapy (marrow leukemic index, MLI) provided the greatest differences between second-line CR and resistant patients. Finally, peroxidase positivity and MLI predicted for remission duration and overall survival. Allogeneic BMT, however, appeared the most important factor for survival and event-free survival of remitting patients. These results are of importance when considering that better defined prognostic factors provide an objective rationale for selecting appropriate strategies for the treatment of patients who do not respond to a single course of induction regimen.

Acute Disease↗

BAVC regimen and autograft for acute myelogenous leukemia in second complete remission.

Since 1984 we have autografted a total of 60 patients with AML in second complete remission (CR) utilizing the BAVC (BCNU, amsacrine, vepesid, cytosine-arabinoside) conditioning regimen and unpurged marrow. Projected disease-free survival (DFS) probability in 42% at 10 years. Autografting was performed at a median interval of 2 months (range 1-13) from second CR. The median duration of first CR was 14 months (range 1-43) and lasted < or = 12 months in 27/60 patients. Three early deaths (5%) occurred, 30 patients relapsed after a median of 6 months from transplant (range 2-28) and, of the remaining 27 patients, 26 are in continuous CR (CCR) after a median follow up of 60 months (range 6-122), while the last patient committed suicide 7 years after ABMT when she was still in CCR. A first CR duration > 12 months is correlated with a significantly better overall survival probability (61 vs 25%, P = 0.02), while no factors influence DFS. Outcome of patients who relapsed after autografting has been analyzed separately; a longer overall survival after relapse is correlated with a longer duration of the second CR (62% at 34 months for patients who relapsed after > 12 months from the autograft vs 5% for the others, P = 0.001). These results confirm that AML patients autografted in second CR with BAVC regimen and unpurged marrow have the possibility of becoming long-term DFS and can therefore be cured.

Adolescent↗

Is aggressive chemotherapy the best choice for patients with acute nonlymphocytic leukemia after myelodysplastic syndromes?

Myelodysplastic syndromes (MDS) evolve in overt acute nonlymphocytic leukemia (ANLL) in about 40% of patients: the treatment of ANLL-MDS is not yet well clarified. To identify the role for aggressive and conservative approaches in ANLL-MDS, we evaluated retrospectively 78 patients in a 7-year period. Thirty-one patients (16 males and 15 females, median age 57.5 years, median MDS duration 5.5 months) were eligible for aggressive chemotherapy; 17 patients (54.8%) achieved complete remission (CR), 10 (32.3%) were resistant and 4 (12.9%) died during induction from infective complications. All patients that achieved CR relapsed, with a median CR duration of 6 months (range 2-28 months); median survival of the whole group was 8.5 months, while median survival of responders was 9 months. No prognostic factor revealed a statistical significance in the outcome, due to the small number of patients in each subgroup. Forty-seven patients (27 male and 20 female, median age 71.8 years, median MDS duration 10.1 months) were not eligible for aggressive chemotherapy; 16 patients (34.2%) received supportive care only, 31 patients (65.8%) needed conservative chemotherapy for disease progression. Median survival of the conservatively treated group was 5.5 months, without statistical difference from the aggressively treated group; 10/47 conservatively treated patients (21%) survived for longer than 12 months. In conclusion, aggressive chemotherapy may play a role only in a selected population of ANLL-MDS patients, while further studies could be helpful to identify the optimal conservative approach.

Adult↗

The role of biotherapies (interleukins, interferons and erythropoietin) in multiple myeloma.

During the last decade, the availability of large numbers of cytokines and growth factors has greatly favoured the use of biotherapies in several haematological disease. For MM, the majority of clinical studies have dealt with the use of IFN-alpha. From these studies it appears that IFN-alpha has a definite role in the treatment of MM especially in the setting of minimal residual disease, as maintenance therapy after response to conventional therapies or HDC followed by BMT procedures or PBSCI. Data on the use of EPO have consistently demonstrated the role of this growth factor in ameliorating the grade of anaemia as well as the quality of life of those MM patients whose disease is complicated by the presence of a severe or moderate anemia. Despite the large amount of experimental data indicating a role for IL-2 and IL-6 in controlling tumour growth, there are only a few clinical studies dealing with their use in MM. From these, it appears that IL-2 and anti-IL-6 antibodies should be further investigated as therapeutic tools useful in maintaining responses, because results show that they arrest tumour progression rather than aid, tumour regression. Finally, in the next years, there will be a wider diffusion of biotherapies in MM that should take into account the roles that IL-1 beta and TNF alpha play in myeloma cell proliferation and bone destruction and the finding that retinoic acid is capable of inhibiting the growth of human myeloma cells in vitro through modulation of IL-6 and its receptor.

Antibodies, Monoclonal↗

Solitary plasmacytoma of bone and extramedullary plasmacytoma: two different entities?

BACKGROUND: The objective of this study was to evaluate the similarities between solitary plasmacytoma of bone (SPB) and extramedullary plasmacytoma (EP). PATIENTS AND METHODS: The clinical features, treatment and survival of 54 patients with localized plasmacytoma (LP) 1990 were carefully reviewed. Follow-up was expected to continue until June 1993. RESULTS: Thirty-two patients were classified as having SPB and 22 EP. Most of the patients were males. particularly those in the SPB group. Their median age at diagnosis was 54 years and no significant difference between the two groups was observed. SPB occurred most frequently in the vertebral column (42%) and EP in the upper respiratory tract (73%). Fifteen patients with SPB and 2 with EP had paraproteinemia at diagnosis, multiple myeloma (MM) became evident in 75% of the patients with persistent paraprotein after therapy, and in only 22% of those in whom it disappeared. Four patients in the SPB group had immunoparesis, and 3 developed MM. Disease progression toward MM was significantly different (p=0.003) in the two groups, while overall survival differences were not significant (p=0.07) unless unrelated causes were excluded (p=0.02). Adjuvant chemotherapy did not seem to limit diffusion of the disease. CONCLUSIONS: Although EP and SPB are both localized forms of plasma cell dyscrasias, SPB seems to have a greater tendency to progression MM. It appears, however, that the apparent stronger propensity of SPB to progress is actually due to the great number of cases that at diagnosis conceal an occult MM.

Adult↗

Combined cytogenetic, FISH and molecular analysis in acute promyelocytic leukaemia at diagnosis and in complete remission.

This study reports the results of a simultaneous application of cytogenetic fluorescence in situ hybridization (FISH) and molecular analysis (RT-PCR) in 28 APL cases (23 M3 and five M3v; 26 studied at diagnosis and two at relapse). FISH on metaphases identified the t(15;17) in all cases who were positive for the PML/RAR alpha transcript by RT-PCR. Conventional cytogenetics revealed the t(15;17) in only 68% of cases. However, it enabled the detection of additional chromosome changes in five cases, three of whom were M3v. 11 patients were also investigated during complete remission (CR) by both FISH and RT-PCR, in order to evaluate residual disease; the duration of CR at the time of analysis ranged between 1 and 16 months, with three patients being studied twice. Comparison of RT-PCR and FISH results showed a very good correlation. In fact, of the 10 samples which were RT-PCR positive for residual disease, all were also recognized by interphase FISH, and eight were positive by metaphase FISH. Of the three samples negative at RT-PCR, all were also negative at the interphase FISH. The results of this study indicate that: (a) the t(15;17) is present in all cases positive for the PML/RAR alpha rearrangement, thus in virtually all true APLs; (b) standard cytogenetics, capable of unravelling the t(15;17) in only 68% of cases, enables recognition of additional chromosome changes of potential clinical and prognostic significance; (c) FISH on interphase nuclei is a reliable tool for the monitoring of residual disease, with a sensitivity greater than that of FISH on metaphase cells and superimposable to that of RT-PCR.

Adolescent↗

Acute myeloid leukemias M2 potentially misdiagnosed as M3 variant French-American-Britain (FAB) subtype: a transitional form?

From 1990 to 1994, 3 patients with de novo acute myeloid leukemia (AML) in whom light microscopy and cytochemistry suggested a FAB subtype M3 variant were observed at our Institute. Immunophenotype showed HLA-DR-, CD13+, CD33+, CD2+, CD9+; promyelocytic features were also detected by electron microscopy. However, leukemic cells lacked both translocation t(15;17) and RAR alpha/PML genes rearrangement. These cases were considered to be 'M2 atypical' subtypes and they contribute to point out how cytogenetics and molecular biology are mandatory for a correct diagnosis of acute promyelocytic leukemia (APL) particularly because therapy with all trans retinoic acid (ATRA) is now the best treatment for APL. Nevertheless these 3 cases indicate that the atypical M2 subtype may be confused with the M3v if only cytochemistry, immunophenotype and electron microscopy are used in the defining the FAB subtypes.

Adult↗