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Transfusion Independence

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Andy Battaglia:
I would like to hear from patients on this subject. For those who have gone from a high iron load to a low iron load that is under control through chelation, has there been any changes in your Hb levels or transfusion frequencies?

Lena:
No, none whatsoever! :nope
In fact it is the opposite that happens: the fewer the transfusions, the lower the body iron - provided you fully comply. Anyway, my ferritin has been maintained in a low level for years now but I have not noticed any difference in B/T I receive. I have not noticed that in my thal friends either. No, I think there is no connection between them two. Don't you think that if there was a connection, doctors would use it to urge patients to chelate?

Lena

ironjustice:

--- Quote from: ironjustice on March 06, 2010, 02:47:02 AM ---This seems to be another article about transfusion requirement going DOWN by chelation of iron.

Iron chelation therapy associated with improvement of hematopoiesis in transfusion-dependent patients
Transfusion


--- End quote ---

This would be another 'human model' of becoming transfusion independent.

Red Blood Cell Transfusion Independence Following the Initiation of
Iron Chelation Therapy in Myelodysplastic Syndrome.
Blood (ASH Annual Meeting Abstracts) 2009 114: Abstract 4844
Maha A Badawi, MD*,1, Linda M Vickars, MD2, Jocelyn M Chase, MD*,1 and
Heather A Leitch, MD, PhD2
1 Medicine, University of British Columbia, Vancouver, BC, Canada,
2 Hematology, St. Paul's Hospital, University of British Columbia,
Vancouver, BC, Canada

Abstract 4844


Background: Iron chelation therapy (ICT) is often used to treat iron
overload (IOL) in patients (pts) requiring transfusion of red blood
cells (RBC) for chronic anemia. In myelodysplastic syndrome (MDS),
guidelines recommend consideration of ICT in pts with lower risk
International Prognostic Scoring System (IPSS) and IOL as defined by a
ferritin level >1000 ug/l; IOL related organ dysfunction; or receipt
of 20 RBC units. During treatment of a pt with MDS and IOL with ICT,
RBC transfusion requirement (TR) ceased. Here we report his course and
review reported cases of RBC transfusion independence (TI) or
decreased RBC TR in MDS pts receiving ICT.


Methods: The pt chart was reviewed and reported cases identified by
PubMed search using the terms ‘MDS’ and ‘iron chelation’. The clinical
characteristics and course of published cases were summarized.


Case: A 76 year (y) old man was referred in May 2004 for management of
MDS diagnosed in 1997, when the white blood cell (WBC) count was 2.4
x109/l; neutrophils, 0.7 x109/l; hemoglobin (Hb), 133 g/l; platelets,
108 x109/l. Bone marrow aspiration and biopsy showed refractory anemia
(RA), karyotype analysis 46,X,-Y,+8, and the IPSS score was
intermediate-1. The erythropoitin (epo) level was 148.3 mIU/ml and the
stem cell assay showed no epo-independent colony growth. In 2004 the
Hb dropped to 60 g/l prompting the initiation of RBC transfusion
support. He required 3 RBC units every 4 weeks to maintain a Hb >90 g/
l and complained of fatigue and functional limitation. Creatinine,
bilirubin, TSH, reticulocyte count, B12 and folate levels were all
normal. The ferritin level in 2004 was 1293 ug/l and 2197 ug/l in
2006. He declined ICT with deferoxamine (DFO) but in 2006 accepted
deferasirox (DFX). He required several dose interruptions and
adjustments for renal insufficiency; the current dose is 5mg/kg/d with
a normal creatinine. Two months (mo) after starting ICT, the Hb
increased spontaneously to 109 g/l and he has not required RBC
transfusion since. The mean Hb since starting ICT was 122 g/l and the
ferritin decreased to 1082 ug/l in 2009. The most recent neutrophil
count was 3.5 x109/l, platelets consistently clump and the MCV is
unchanged at 120 fl. He reports excellent energy and an improved
quality of life, and has remained clinically well and RBC transfusion
independent to the present, 36 mo from the initiation of ICT.


Literature review: There are 18 published cases of MDS showing
improvement in Hb with ICT; 9 became RBC transfusion independent.
Characteristics of the 10 TI pts were: median age at MDS diagnosis 58
(range 18-74) y; male, n=5. MDS subtype: RA, n=5; RARS, n=2, RCMD,
n=1; RAEB, n=2. IPSS (reported in 8): low, n=1; int-1, n=5; int-1 or
2, n=1; high, n=1. ICT was: DFO, n=7; DFX, n=3. Median time to RBC TI
was 17.5 (1-24) mo and TI duration 13 (3-28) mo to date. Of pts who
had decreased RBC transfusion requirements with ICT but did not
achieve transfusion independence: median age (reported in 3) was 67
(45-78) y; gender (reported in 3) female, n=3; MDS subtype: RA, n=8;
RAEB-t, n=1; IPSS: int-1, n=3; ICT: DFO, n=8; DFX, n=1. Median time to
decreased TR was 14.4 (3-24) mo; median duration of decreased TR
(reported in 3) 9 (6-32) mo; initial TR 50.9 (19.7-447) g Hb/mo;
median decrease in TR 12.7 (0.1-88) g Hb/mo. In one report of 6 pts, 2
with pancytopenia showed improvement with ICT in WBC from 1.4 to 1.9
x109/l (p<0.0001) and neutrophils from 0.51 to 0.94 x109/l (p<0.001).
The platelet count increased from 16.6 to 22.5 x109/l (p<0.001) and
14.6 to 29.6 x109/l (p<0.00001) within 3 mo and the MCV decreased
significantly in 5 by a mean of 5.1 (2.1-11.7) fl, normalizing in 2.
In a second report, neutrophils increased in 8 of 9 pts; in 4 the
initial neutrophil count was <1 x109/l, and platelet counts increased
in 7 of 11 pts, in 4 the initial platelet count was <20 x109/l.


Conclusions: In summary, our pt is the 19th patient with MDS reported
to date in whom improved Hb followed the initiation of ICT; 9 had a
decrease in RBC transfusion requirements, and RBC transfusion
independence occurred in 10. The remarkable course of these pts adds
to evidence that ICT may be of clinical benefit for selected patients
with MDS and IOL. Although the improvement in WBC and platelet counts
with ICT in some pts implies a suppressive effect of IOL on
hematopoiesis that may be abrogated by ICT, the mechanism by which the
effects of ICT on transfusion requirements occur, and the frequency
with which they occur, remains an area for future investigation.


Disclosures: Off Label Use: This presentation discusses the use of
iron chelation therapy deferoxamine and deferasirox in patients with
myelodysplastic syndrome.. Vickars: Novartis Canada: Honoraria,
Research Funding. Leitch: Novartis Canada: Honoraria, Research
Funding, Speakers Bureau.


© 2009 American Society of Hematology

nice friend:

--- Quote ---For those who have gone from a high iron load to a low iron load that is under control through chelation, has there been any changes in your Hb levels or transfusion frequencies?
--- End quote ---
yes , there's a big change in everything related to me and my health after getting Fe within the safe range. ....  ii never seen my pre-tx Hb around 11 in my whole life but xperienceed that twice in last 2 months .... i dont know ther reason and the reality of it ... but i think Vhelation and managin  Fe within a safe range shows positive effects on ur health in many ways ....

Umair

Dori:

--- Quote from: ironjustice on February 17, 2010, 06:16:46 PM ---This seems to say there is an "unrecognized" iron load in some with thalassemia.
Again pointing to a possibility of iron PREdisease as evidenced by the use of an iron chelating substance Hp70 to "reverse genetic disease" .. "In particular, they
found that drugs called proteosome inhibitors could provide one way of
manipulating cells into producing more of a so-called chaperone
protein, named Hsp70, which helps amino acid chains fold into their
proper protein form." ..?

Iron overload in thalassaemia intermedia: reassessment of iron chelation strategies
Authors: Taher, Ali1; Hershko, Chaim2; Cappellini, Maria Domenica3
Source: British Journal of Haematology, Volume 147, Number 5,
December 2009 , pp. 634-640(7)
Publisher: Blackwell Publishing

Abstract:

Summary

Thalassaemia intermedia (TI) is a syndrome marked by its diverse
underlying genetic basis although its pathophysiology remains
unclear, particularly regarding the nature of iron loading and toxicity.
It is, however, evident that there are key differences from the
extensively studied thalassaemia major (TM) population and
caution is required when assessing iron load based on serum
ferritin values, as this approach is known to underestimate the true
extent of iron loading in patients with TI.
Although effective iron chelation therapy has been available for
many years, studies in TI-specific populations are rare and evidence
suggests that management of iron levels may be less rigorous than
in patients with TM and other chronic anaemias.
Better understanding of the need to assess and treat iron overload
in both transfused and non-transfused TI patients is clearly required.
Keywords: thalassaemia intermedia; iron overload; iron chelation

Document Type: Research article

DOI: 10.1111/j.1365-2141.2009.07848.x

Affiliations: 1: American University of Beirut, Beirut, Lebanon 2:
Hebrew University of Jerusalem, Jerusalem, Israel 3:
Università di Milano, Policlinico Foundation IRCCS,
Milan, Italy
 

--- End quote ---

Ali Taher is a famous doctor in the Thal world. I've met him in Berlin but I'd no chance to ask him my questions.
I haven't read your article yet. In which databank do you look??

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