Clinical Features of DIC According to the French-american-british (FAB) Classification in Patients With Acute Leukemia and Thrombomodulin Alfa Treatment – Cross-sectional Analysis of a Post-marketing Surveillance Study Cohort –

2021 ◽  
Author(s):  
Yoshinobu Seki ◽  
Goichi Honda ◽  
Noriaki Kawano ◽  
Toshimasa Uchiyama ◽  
Kazuo Kawasugi ◽  
...  
Keyword(s):  
Acute Leukemia ◽  
Clinical Features ◽  
Lymphoblastic Leukemia ◽  
Study Cohort ◽  
Cross Sectional ◽  
Safety And Efficacy ◽  
Post Marketing Surveillance ◽  
Fab Classification ◽  
Post Marketing ◽  
French American British

Abstract Background: The aims of this study were to analyze the clinical features of a large number of cases with disseminated intravascular coagulation (DIC) associated with acute leukemia, and to clarify the safety and efficacy of thrombomodulin alfa (TM-α) using the French-American-British (FAB) classification of hematological malignancies.Methods: We retrospectively examined 644 patients with acute leukemia in post-marketing surveillance for TM-α.Results: M3, M2, M4, M1, and M5 subtypes of acute myeloid leukemia (AML), and L2 and L1 subtypes of acute lymphoblastic leukemia (ALL) have been found more frequently among patients with DIC. Bleeding symptoms at baseline were more frequent in M3 and M7 subtypes. Fibrinogen concentrations were lower and plasmin-plasmin inhibitor complex values were higher in M3 and Philadelphia-positive (Ph+) ALL. Overall DIC resolution rate was 60.2% with higher rates in L1 and Ph+ ALL, lower in M1, and generally higher in ALL than in AML. The overall survival rate was 79.8%, generally high, with higher survival rates in L3, Ph+ ALL, and M3. In M3 and M7, with high frequencies of pre-existing bleeding, TM-α improved bleeding symptoms. Post-administration DIC scores in each subtype were significantly improved compared with pre-administration scores, except in M6, M7, and MDS-overt AML.Conclusions: This study showed the clinical features of DIC associated with acute leukemia among FAB classifications and also elucidated the safety and efficacy profiles of TM-α by detailed classification based on the FAB classification in clinical practice.Trial registration: The clinical characteristics and treatment outcomes of patients with DIC treated with TM-α between May 2008 and April 2010 were retrospectively analyzed by subgroup analysis of post-marketing surveillance data.

scholarly journals Simultaneous or sequential expression of lymphoid and myeloid phenotypes in acute leukemia

Blood ◽  
1985 ◽  
Vol 65 (1) ◽  
pp. 142-148 ◽  
Author(s):  
PB Neame ◽  
P Soamboonsrup ◽  
G Browman ◽  
RD Barr ◽  
N Saeed ◽  
...  
Keyword(s):  
Acute Leukemia ◽  
Myeloid Leukemia ◽  
Chromosomal Abnormalities ◽  
Lymphoblastic Leukemia ◽  
Differentiation Markers ◽  
Lymphoid Leukemia ◽  
Chromosome Marker ◽  
Fab Classification ◽  
Blast Cells ◽  
French American British

Abstract Acute mixed myeloid-lymphoid leukemia is uncommon. We report four cases in which myeloid and lymphoid cell markers were observed simultaneously or sequentially when 94 patients with acute leukemia were phenotyped according to the French-American-British (FAB) classification system, with cytochemical stains, and with immunologically defined differentiation markers (identified by monoclonal antibodies and antiterminal deoxynucleotidyl transferase [TdT]). In one case, conversion from acute lymphoblastic leukemia to acute myeloid leukemia was noted (FAB L1, TdT+ to FAB M4, Auer rods, TdT-). In another patient, two distinct populations of myeloid and lymphoid blast cells were observed simultaneously (TdT-, LeuM1+/TdT+, LeuM1-). In two additional patients, acute leukemia was characterized by the expression of both lymphoid and myeloid markers on the same cell (TdT+/Leu M1+, B4+/Leu M1+ and greater than or equal to 70% TdT+, T11+, My9+). The Philadelphia (Ph1) chromosome was negative in all cases, though other chromosomal abnormalities were noted in three out of four cases. Malignant transformation of a pluripotential stem cell for both lymphoid and myeloid lineages, with or without the Ph1 chromosome marker, could explain the coexistence of distinct populations of lymphoblasts and myeloblasts in acute leukemia. Acute leukemia with a biphenotypic profile may reflect genome depression accompanying neoplasia.

scholarly journals Clinical Features of Disseminated Intravascular Coagulation According to the French-American-British Classification in Patients With Acute Leukemia and Thrombomodulin Alfa Treatment—A Cohort Study Using a Postmarketing Surveillance Database

2021 ◽  
Vol 27 ◽  
pp. 107602962110540
Author(s):  
Seki Yoshinobu ◽  
Goichi Honda ◽  
Noriaki Kawano ◽  
Toshimasa Uchiyama ◽  
Kazuo Kawasugi ◽  
...  
Keyword(s):  
Acute Leukemia ◽  
Clinical Features ◽  
Disseminated Intravascular Coagulation ◽  
Lymphoblastic Leukemia ◽  
Postmarketing Surveillance ◽  
Intravascular Coagulation ◽  
Plasmin Inhibitor ◽  
Surveillance Database ◽  
French American British

The aims of this study were to analyze the clinical features of a large number of cases with disseminated intravascular coagulation (DIC) associated with acute leukemia and to assess the safety and efficacy of thrombomodulin alfa (TM-α) using the French-American-British (FAB) classification of hematological malignancies. We retrospectively examined 644 patients with acute leukemia in postmarketing surveillance for TM-α. M3, M2, M4, M1, and M5 subtypes of acute myeloid leukemia (AML) and L2 and L1 subtypes of acute lymphoblastic leukemia (ALL) have been found more frequently among patients with DIC. Bleeding symptoms at baseline were more frequent in M3 and M7 subtypes. Fibrinogen concentrations were lower, and plasmin-plasmin inhibitor complex values were higher in M3 and Philadelphia-positive (Ph+) ALL. Overall DIC resolution rate was 60.2%, higher in L1 and Ph+ ALL, lower in M1, and generally higher in ALL than in AML. Overall survival rate was generally high, at 79.8%, with higher rates in L3, Ph+ ALL, and M3. Regardless of FAB subgroup, TM-α showed improved bleeding symptoms and DIC scores in clinical practice for DIC patients with acute leukemia.

scholarly journals Classifying acute leukemia by immunophenotyping: a combined FAB- immunologic classification of AML

Blood ◽  
1986 ◽  
Vol 68 (6) ◽  
pp. 1355-1362 ◽  
Author(s):  
PB Neame ◽  
P Soamboonsrup ◽  
GP Browman ◽  
RM Meyer ◽  
A Benger ◽  
...  
Keyword(s):  
Monoclonal Antibodies ◽  
Acute Lymphoblastic Leukemia ◽  
Acute Leukemia ◽  
Gold Standard ◽  
Lymphoblastic Leukemia ◽  
Myelogenous Leukemia ◽  
Fab Classification ◽  
Acute Myelogenous ◽  
French American British

Abstract A panel of commercially available monoclonal antibodies and five heteroantisera were used to distinguish and subtype 138 cases of acute leukemia (AL). The immunophenotype was compared with the French- American-British (FAB) classification obtained on the cases. The immunophenotype discriminated acute myelogenous leukemia (AML) from acute lymphoblastic leukemia (ALL) and recognized cases not distinguished by cytochemistry (22% of cases), mixed lineage phenotypes (13% of cases), and cases with separate populations of lymphoblasts and myeloblasts (one case). Using the immunologic panel and derived criteria to subtype AML, correspondence of the immunophenotype to the FAB subtypes M1, M2, M4, and M5 was possible in greater than 80% of cases. A combined classification of the immunophenotype and FAB morphology/cytochemistry was devised for AML subtyping. It is recommended that immunophenotyping should be done at least in all cases with negative orinconclusive cytochemistry. At present, we suggest that until a “gold standard” for identifying leukemic subtypes is developed, the best method for typing acute leukemia is by using a combination of morphology, cytochemistry and immunophenotyping.

scholarly journals Pattern of childhood acute leukemia presentation at a tertiary hospital in Nigeria: a five-year review

2018 ◽  
Vol 5 (6) ◽  
pp. 2123
Author(s):  
Adewumi B. Oyesakin ◽  
Vincent E. Nwatah ◽  
Nwankwo U. Ukpai ◽  
Ekaette I. David ◽  
Tamunomieibi T. Wakama ◽  
...  
Keyword(s):  
Acute Lymphoblastic Leukemia ◽  
Acute Leukemia ◽  
Clinical Features ◽  
Childhood Leukemia ◽  
Lymphoblastic Leukemia ◽  
Tertiary Hospital ◽  
Cross Sectional ◽  
Acute Leukemias ◽  
Lost To Follow Up

Background: Acute leukemia is the most common childhood malignancy but its occurrence in low- and middle-income countries are under-reported. Its pattern of presentation varies depending on several factors. The objective of this report is to determine the pattern of presentation of acute leukemias in children at a tertiary hospital in Nigeria.Methods: A retrospective cross-sectional study of children managed for acute leukemia at the Paediatric Department in a 5-year period. Of 31 patients, 27 had adequate records, which were reviewed. Data collected include patient’s demographics, clinical features and treatment outcome.Results: There were 16 males and 11 females, aged 8 months to 16 years (mean 7.45 years ±4.75 SD). The pattern of clinical features were fever (85.2%), pallor (92.6%) and splenomegaly (51.9%). The specific leukemia type ratio for Acute Myeloid leukemia (AML) and Acute lymphoblastic leukemia (ALL) was 1: 2.9. The parents of three patients took their children away before commencement of treatment, one patient completed treatment and 6 (22.2%) died before completing treatment. Nearly half of the patients were lost to follow up to seek alternative care while 9 (33.3%) of the patients were in remission at last follow up. Lost to follow-up was found not to be significantly associated with socioeconomic status, age and sex respectively.Conclusions: Acute lymphoblastic leukemia remains the predominant type of childhood leukemia in our setting. Majority of the patients presented with fever and pallor moreover the default to follow-up plagues treatment completion.

scholarly journals Simultaneous or sequential expression of lymphoid and myeloid phenotypes in acute leukemia

Blood ◽  
1985 ◽  
Vol 65 (1) ◽  
pp. 142-148 ◽  
Author(s):  
PB Neame ◽  
P Soamboonsrup ◽  
G Browman ◽  
RD Barr ◽  
N Saeed ◽  
...  
Keyword(s):  
Acute Leukemia ◽  
Myeloid Leukemia ◽  
Chromosomal Abnormalities ◽  
Lymphoblastic Leukemia ◽  
Differentiation Markers ◽  
Lymphoid Leukemia ◽  
Chromosome Marker ◽  
Fab Classification ◽  
Blast Cells ◽  
French American British

Acute mixed myeloid-lymphoid leukemia is uncommon. We report four cases in which myeloid and lymphoid cell markers were observed simultaneously or sequentially when 94 patients with acute leukemia were phenotyped according to the French-American-British (FAB) classification system, with cytochemical stains, and with immunologically defined differentiation markers (identified by monoclonal antibodies and antiterminal deoxynucleotidyl transferase [TdT]). In one case, conversion from acute lymphoblastic leukemia to acute myeloid leukemia was noted (FAB L1, TdT+ to FAB M4, Auer rods, TdT-). In another patient, two distinct populations of myeloid and lymphoid blast cells were observed simultaneously (TdT-, LeuM1+/TdT+, LeuM1-). In two additional patients, acute leukemia was characterized by the expression of both lymphoid and myeloid markers on the same cell (TdT+/Leu M1+, B4+/Leu M1+ and greater than or equal to 70% TdT+, T11+, My9+). The Philadelphia (Ph1) chromosome was negative in all cases, though other chromosomal abnormalities were noted in three out of four cases. Malignant transformation of a pluripotential stem cell for both lymphoid and myeloid lineages, with or without the Ph1 chromosome marker, could explain the coexistence of distinct populations of lymphoblasts and myeloblasts in acute leukemia. Acute leukemia with a biphenotypic profile may reflect genome depression accompanying neoplasia.

scholarly journals Classifying acute leukemia by immunophenotyping: a combined FAB- immunologic classification of AML

Blood ◽  
1986 ◽  
Vol 68 (6) ◽  
pp. 1355-1362
Author(s):  
PB Neame ◽  
P Soamboonsrup ◽  
GP Browman ◽  
RM Meyer ◽  
A Benger ◽  
...  
Keyword(s):  
Monoclonal Antibodies ◽  
Acute Lymphoblastic Leukemia ◽  
Acute Leukemia ◽  
Gold Standard ◽  
Lymphoblastic Leukemia ◽  
Myelogenous Leukemia ◽  
Fab Classification ◽  
Acute Myelogenous ◽  
French American British

A panel of commercially available monoclonal antibodies and five heteroantisera were used to distinguish and subtype 138 cases of acute leukemia (AL). The immunophenotype was compared with the French- American-British (FAB) classification obtained on the cases. The immunophenotype discriminated acute myelogenous leukemia (AML) from acute lymphoblastic leukemia (ALL) and recognized cases not distinguished by cytochemistry (22% of cases), mixed lineage phenotypes (13% of cases), and cases with separate populations of lymphoblasts and myeloblasts (one case). Using the immunologic panel and derived criteria to subtype AML, correspondence of the immunophenotype to the FAB subtypes M1, M2, M4, and M5 was possible in greater than 80% of cases. A combined classification of the immunophenotype and FAB morphology/cytochemistry was devised for AML subtyping. It is recommended that immunophenotyping should be done at least in all cases with negative orinconclusive cytochemistry. At present, we suggest that until a “gold standard” for identifying leukemic subtypes is developed, the best method for typing acute leukemia is by using a combination of morphology, cytochemistry and immunophenotyping.

scholarly journals All-case Japanese post-marketing surveillance of the real-world safety and efficacy of rituximab treatment in patients with refractory nephrotic syndrome

2021 ◽  
Author(s):  
Mana Kobayashi ◽  
Yutaro Kageyama ◽  
Takashi Ando ◽  
Junko Sakamoto ◽  
Shohji Kimura
Keyword(s):  
Nephrotic Syndrome ◽  
Standard Deviation ◽  
Real World ◽  
Pediatric Patients ◽  
Safety And Efficacy ◽  
The Real ◽  
Post Marketing Surveillance ◽  
Post Marketing ◽  
Refractory Nephrotic Syndrome ◽  
Observation Period

Abstract Background Rituximab is conditionally approved in Japan for use in patients with refractory nephrotic syndrome. To meet the conditions of approval, an all-case post-marketing surveillance study was conducted to confirm the real-world safety and efficacy of rituximab in patients of all ages with refractory nephrotic syndrome. Methods All patients scheduled to receive rituximab treatment for refractory nephrotic syndrome were eligible to register (registration: August 29, 2014 through April 15, 2016); the planned observation period was 2 years from the initiation of rituximab treatment (intravenous infusion, 375 mg/m2 once weekly for four doses). The study was conducted at 227 hospitals throughout Japan. Adverse drug reactions (ADRs) were collected for safety outcomes. The efficacy outcomes were relapse-free period and the degree of growth in pediatric (< 15 years) patients. Results In total, 997 (447 pediatric) patients were registered; 981 (445) were included in the safety analysis set; 852 (402) completed the 2-year observation period; and 810 (429) were included in the efficacy analysis set. Refractory nephrotic syndrome had developed in childhood for 85.0% of patients, and 54.6% were aged ≥15 years. ADRs were observed in 527 (53.7%) patients, treatment-related infection/infestation in 235 (24.0%) patients, and infusion reactions in 313 (31.9%) patients. The relapse-free period was 580 days (95% confidence interval, 511–664). There was a significant change in height standard deviation score (pediatric patients; mean change, 0.093; standard deviation, 0.637; P = 0.009). Conclusion The safety and efficacy of rituximab treatment in patients with refractory nephrotic syndrome were confirmed in the real-world setting. Clinical trial registration UMIN000014997.

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