Tuesday, September 16, 2014

Interesting case at CRBCM: Acute Myeloid Leukemia with myelomonocytic differentiation in a patient with possible Lupus

This is a 74 YOHM who presented to Sierra Providence with vomiting, nausea , poor appetite and weight loss, he was found with a severe anemia. A bone marrow biopsy obtained on 08/11/2014 suggested a Hypercellular Marrow involved with Acute myelogenous leukemia with myelomonocytic differentiation.  There was 70 to 80 % of myeloblasts by morphology and flow.  Further cytogenetic studies revealed a positive inversion 16 ( MYH1) with 7q- (DS486). The CBFB was found in 90% of cells and RUNX1-I mutation in 90% of cells. 
Of note: The ANA was positive.   HIV negative.
The patient received a course of hydroxyurea prior to a standard 7+3 induction therapy with Daunarubicin and ARA-C. A new bone marrow biopsy obtained on 09/02/2014 suggests residual disease with 3% blasts 10 to 15% myelomonoblasts.  the patient will need a reinduction therapy with Idarubicin and  ARA-C.
 Prior to this admission, the patient, an old teacher, was otherwise healthy. He has no history of hypertension, diabetes or asthma.
Of interest: The patient's sister has an open Lupus.

This case is very interesting as it suggests progression of Lupus into a monocytic AML
did RUNX-1 under various pressure go awry,
did presence of RUNX-1 suggest activity of Etoposide or may be just activity of Decitabine.
but this is an inversion 16 case, should we forgo standard induction because of the patient age?  But he did tolerate induction treatment at the local Hospital.   He had the 7q minus deletion and therefore a bad prognosis indicator mitigating the Inv(16) presence.  Could he have transplant in good hands...the man has a good performance status and flew through his first attempt to induction!
your feed back appreciated! 

Ida will do (
"Idarubicin /ˌdəˈrbɨsɪn/ or 4-demethoxydaunorubicin is an anthracycline antileukemic drug. It inserts itself into DNA and prevents DNA from unwinding by interfering with the enzyme topoisomerase II. It is an analog of daunorubicin, but the absence of a methoxy group increases its fat solubility and cellular uptake.[1] Similar to other anthracyclines, it also induces histone eviction from chromatin.[2]"  wikipedia
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