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Category: Mini Review NHL

Plasmablastic Lymphoma or Plasmablastic Myeloma ?

February 27, 2022February 28, 2022 Hem Updates

A 27 year old male presented with pain in the left scapular region x 1 months. On examination 2 x 2 cm left axillary node was noted. No hepatosplenomegaly and no other palpable peripheral nodes were seen. Relevant Blood parameters Read More …

Malignant Hematology, Mini Review NHL, NHLsPlasmablastic Lymphoma, Plasmablastic MyelomaLeave a comment

Management of EMZL of the ocular adnexa – Mini Review

October 31, 2021November 1, 2021 Hem Updates

Ocular adnexal Lymphomas comprise approximately 1% to 2% of non-Hodgkin lymphomas (NHLs) and 8% of extranodal lymphomas. The most common subtype, accounting for up to 80% of cases of primary ocular adnexal lymphoma (POAL), is marginal zone lymphoma of mucosa-associated Read More …

Malignant Hematology, Mini Review NHL, NHLsAdnexal Lymphoma, EMZL of the ocular adnexa, Marzinal Zone LymphomaLeave a comment

Extranodal NK/T Cell Lympoma, Nasal Type – Mini Review

August 31, 2021October 31, 2021 Hem Updates

Author: Sujeet Kumar @sujeethemat Some Salient points:- [1] Approximately 70-75% cases are diagnosed in Limited stage (Stage I and II) [2] Median Age at presentation = 50 years [3] Delition 6q21 is MC cytogenetic aberation. [4] Presence of EBV is Read More …

Malignant Hematology, Mini Review NHL, NHLsExtranodal NK/T Cell LympomaLeave a comment

Rituximab Maintenance in NHLs – Mini Review

August 30, 2021September 8, 2021 Hem Updates

Author: Sujeet Kumar @sujeethemat Rituximab is given as maintenance therapy in NHLs, although benefits of maintenance rituximab (MR) are still unclear for many NHLs and regimens and durations are confusing. My attempt to simplify this is here. FOLLICULAR LYMPHOMA [1] Read More …

Malignant Hematology, Mini Review NHL, NHLsCLL, DLBCL, Follicular Lymphoma, maintenance, Mantle cell Lymphoma, NHL, Non Hodgkin's Lymphoma, rituximabLeave a comment

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FDA Approval News

Malignant
BMT
CAR-T CELLS
Benign

25/05/2022: FDA approved ivosidenib in combination with azacitidine (azacitidine for injection) for newly diagnosed AML with IDH1 mutation in adults 75 years or older, or who have comorbidities that preclude use of intensive induction chemotherapy. Approval was based on a randomized, multicenter, double-blind, placebo-controlled study (AG120-C-009, NCT03173248) that included 146 patients with newly-diagnosed AML with an IDH1 mutation who were 75 years or older, or who had comorbidities.

20/05/2022: FDA approved azacitidine for pediatric patients with newly diagnosed juvenile myelomonocytic leukemia (JMML). Efficacy was evaluated in AZA-JMML-001 (NCT02447666), an international, multicenter, open-label study to evaluate the pharmacokinetics, pharmacodynamics, safety, and activity of azacitidine prior to hematopoietic stem cell transplantation (HSCT) in 18 pediatric patients with JMML.

29/10/2021: FDA granted accelerated approval to asciminib (Scemblix, Novartis AG) for patients with Philadelphia chromosome-positive chronic myeloid leukemia (Ph+ CML) in chronic phase (CP), previously treated with two or more tyrosine kinase inhibitors (TKIs), and approved asciminib for adult patients with Ph+ CML in CP with the T315I mutation.

14/9/2021: FDA granted accelerated approval to zanubrutinib for adult patients with relapsed or refractory marginal zone lymphoma (MZL) who have received at least one anti-CD20-based regimen.

1/9/2021: FDA approved zanubrutinib for adult patients with Waldenström’s macroglobulinemia (WM). Zanubrutinib was investigated in ASPEN (NCT03053440), a randomized, active control, open-label trial, comparing zanubrutinib and ibrutinib in patients with MYD88 L265P mutation (MYD88MUT) WM.

16/6/2021: FDA approved avapritinib for adult patients with advanced systemic mastocytosis (AdvSM), including patients with aggressive systemic mastocytosis (ASM), systemic mastocytosis with an associated hematological neoplasm (SM-AHN), and mast cell leukemia (MCL).

24/08/2022: FDA approved ibrutinib for pediatric patients ≥ 1 year of age with cGVHD after failure of 1 or more lines of systemic therapy. Formulations include capsules, tablets, and oral suspension. Efficacy was evaluated in iMAGINE (NCT03790332), an open-label, multi-center, single-arm trial of ibrutinib for pediatric and young adult patients 1 year to less than 22 years old with moderate or severe cGVHD. (NOTE: FDA had already Approved ibrutinib for Chronic Graft Versus Host Disease in adult patients in Aug 2017)

15/12/2021: FDA approved abatacept for the prophylaxis of acute graft versus host disease (aGVHD), in combination with a calcineurin inhibitor (CNI) and methotrexate (MTX), in adults and pediatric patients 2 years of age and older undergoing hematopoietic stem cell transplantation (HSCT) from a matched or 1 allele-mismatched unrelated donor. This is the first drug approved to prevent aGVHD. Approval was based on GVHD-1 (NCT 01743131) trial.

23/11/2021: FDA approved maribavir as the first drug for treating adults and pediatric patients (12 years of age and older and weighing at least 35 kilograms) with post-transplant cytomegalovirus (CMV) infection/disease that does not respond (with or without genetic mutations that cause resistance) to available antiviral treatment for CMV.

22/9/2021: FDA approved ruxolitinib for chronic graft-versus-host disease (cGVHD) after failure of one or two lines of systemic therapy in adult and pediatric patients 12 years and older. Efficacy was evaluated in REACH-3, a randomized, open-label, multicenter clinical trial of ruxolitinib compared to best available therapy (BAT) for corticosteroid-refractory cGVHD after allogeneic stem cell transplantation. (Read more about Ruxolitinib)

16/7/2021: FDA approved belumosudil, a kinase inhibitor, for adult and pediatric patients 12 years and older with chronic graft-versus-host disease (chronic GVHD) after failure of at least two prior lines of systemic therapy.

24/06/2022: FDA approved lisocabtagene maraleucel for adult patients with large B-cell lymphoma (LBCL) who have refractory disease to first-line chemoimmunotherapy or relapse within 12 months of first-line chemoimmunotherapy; or refractory disease to first-line chemoimmunotherapy or relapse after first-line chemoimmunotherapy and are not eligible for hematopoietic stem cell transplantation (HSCT) due to comorbidities or age. It is not indicated for the treatment of patients with primary central nervous system lymphoma. Efficacy was evaluated in TRANSFORM (NCT03575351), a randomized, open-label, multicenter trial in adult patients with primary refractory LBCL or relapse within 12 months of achieving complete response (CR) to first-line therapy.

27/05/2022: FDA granted accelerated approval to tisagenlecleucel for adult patients with relapsed or refractory follicular lymphoma (FL) after two or more lines of systemic therapy. The approval was based on the ELARA trial (NCT03568461), a multicenter, single-arm, open-label trial evaluating tisagenlecleucel, a CD19-directed chimeric antigen receptor (CAR) T cell therapy, in adult patients who were refractory or relapsed within 6 months after completion of two or more lines of systemic therapy (including an anti-CD20 antibody and an alkylating agent) or relapsed after autologous hematopoietic stem cell transplant. Following lymphodepleting chemotherapy, tisagenlecleucel was administered as a single intravenous infusion with a target dose of 0.6 to 6.0 x 108 CAR-positive viable T cells.

1/04/2022: FDA approved axicabtagene ciloleucel (Yescarta, Kite Pharma, Inc.) for adult patients with large B-cell lymphoma (LBCL) that is refractory to first-line chemoimmunotherapy or relapses within 12 months of first-line chemoimmunotherapy. It is not indicated for the treatment of patients with primary central nervous system lymphoma. Approval was based on ZUMA-7 trial.

1/10/2021: FDA approved brexucabtagene autoleucel for adult patients with relapsed or refractory B-cell ALL. Efficacy was evaluated in ZUMA-3 (NCT02614066), a single-arm multicenter trial that evaluated brexucabtagene autoleucel, a CD19-directed chimeric antigen receptor (CAR) T-cell therapy, in adults with relapsed or refractory B-cell precursor ALL.

Created on October 09, 2022

BTK inhibitor

Ibrutinib - an oral Bruton's tyrosine kinase (BTK) inhibitor is currently (oct 2022) not approved for treatment of :-

Mantle Cell Lymphoma with at least one prior therapy.

Waldenström’s Macroglobulinemia

Marginal Zone Lymphoma with at least one prior anti-CD20-based therapy.

Chronic Graft Versus Host Disease in adult and pediatric patients age 1 year and older

DLBCL patients with at least one prior anti-CD20-based therapy.

Ibrutinib is an oral Bruton's tyrosine kinase (BTK) inhibitor for the treatment of mantle cell lymphoma (MCL), chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL), Waldenström’s macroglobulinemia (WM), marginal zone lymphoma (MZL), and chronic graft versus host disease (cGVHD). It is not approved for DLBCL.

All Drug Update Quizes

Created on April 16, 2023

Poll of the Week 7

GATA1

GATA2

CRLF2 gene

RAS gene

Myeloid leukemia in patients with Down syndrome (ML - DS) originates in utero and typically presents with a self-limiting, neonatal leukemic syn drome known as transient abnormal myelopoiesis (TAM) that is caused by cooperation between trisomy 21 – associated abnormalities of fetal hematopoiesis and somatic mutations in N - terminal mutations in the transcription factor GATA1. Around 10 % of neonates with DS have clinical signs of TAM, although the frequency of hematologically silent GATA1 mutations in DS neonates is much higher (~25%).

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Online Calculators In Hematology

	MDS Calculators
1	Online MDS IPSS Calculator
2	Online MDS IPSS-R Calculator
	Coagulation Calculators
1	4T Clinical Score for HIT
2	Wells’ score calculator
3	Diagnostic criteria for APS

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