Myeloid Neoplasm Classification 1. Myeloproliferative neoplasms (MPN) 5 AML: Classification
2016 Classification  AML AML is clinically and biologically heterogenous disease with different morphological and cytogenetic presentations A revision of this WHO 2008 classification is currently underway, specifically regarding underlined item below Additional References: Based on this there is a proposed genomic classification of AML NEJM 2016 What are the 8 Functional Categories of genes that are affected in AML Clonal evolution and patterns of AML. Clinical Relevance of molecular Prognostic factors of AML are
 Anthracyclin for AML induction therapy BM transplant in AML   Newer concepts in AML treatment Epigenetic changes in AML and treatment strategies to target epigenetic changes AML Treatment Complications: Case Discussion 1 JJM 47 yo F is seen for weakness and gum bleeding of 3 days associated with Fever of 101.3 degree F. CBC shows the following. CMP is essentially normal.  What is the DDx?
Diagnosis of AML was made, and not CML due to the presence of blasts of 52 % in peripheral smear. How is AML diagnosed? Presence of blasts > 20 % in periphery or in BM What is the next best step? Determine if it is APML as it is treated. How to do so? PBS  PBS is above shows all blasts with no visible aur rods. Hence, AML not likely APML was diagnosed. What is the next step?
Despite using Hydroxyurea, cell count remained high as follow  On day 4 of admission, Peripheral blood flow cytometry result is as follow Diagnostic of acute myelogenous leukemia, with monocytic differentiation. The blast express CD4 subset, subset CD11b, CD11c, CD13, CD33, subset CD34, CD38, CD45, HLA DR, CD 117 and CD 123 BM Biopsy is also done and sample is sent for path only as flow and cytogenetics were done from peripheral blood Now that diagnosis is made, how should this be treated?
After treatment, on day 3 following is the response. 98 K down from 125 was still an effect of Hydroxyurea, after that is due to Chemo.  After day 3 of treatment, allopurinol was stopped and IV fluid rate was decreased as patient was out of window for Tumor lysis syndrome Cytogentics came as follow Cytogenetics normal 46 X,Y. NPM-1 mutated (favorable), Flt3-ITD positive (unfavorable), Flt3 D835 mutation positive (unfavorable), CEBPA mutation negative, kit mutation negative On day 14 of induction, patient continued to have some fever labs were as follow . Leukine 500 mcg subq daily was started . Day 14 marrow was not done, though had rapid dramatic drop in WBC with treatment initiation ON day 19-20 patient started to have BM recovery, Patient continued to have fever on day 23 requiring BAL to evaluate for pulmonary infection On 11/5, i.e 35 days of induction chemo, BM Bxp was performed, shows CR in BM bxp Another 30 days later, i.e 2 months after the day 1 of induction chemotherapy, patient was admitted again for Consolidation therapy high dose Ara-C 2 g/m2 IVB q12h d 1,3,5 (CALGB style consolidation). Used 2g/m2 rather than 3 g/m2 in light of patient's prior severe mucositis with induction. Another two months later patient was admitted for consolidation # 2 with high-dose araC 2 g/m² IVB every 12 hours days 1, 3, and 5 Another 6 weeks later, patient was admitted for consolidation # 3 midostaurin was planned in light of favorable trial data in FLT-3 abnormal patients . araC 2 g/m² IVB every 12 hours days 1, 3, and 5 was given. 6 weeks later, # 4 consolidation treatment was given After that, patient was given Midostaurin (two 25 mg capsules BID x 14 days 1 year later, patient was readmitted and was found to have relapse; 50% blasts WBC 14.25 HLA-DNA typing was ordered and patient readmitted for reinduction rx. Events summarized to date is as follow  Labs at various stages of treatment and prior to relapse was as follow    After relapse, salvage chemotherapy was started with FLAG-Ida (G-CSF deleted)
Subsequently, patient had chemo and likely steroids induced pancreatitis Subsequently, patient had V tach. Anthracycline-induced cardiomyopathy. EF 30-35% was diagnosed Patient is currently working to get a BM transplant What is the stage for this patient? What is the prognosis. What is the complication of AML and complications during treatment? How to manage them? How should this patient be treated? |
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