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As a german HCL patient I would give my respect to the english version of the Hairy cell article, which is far better than the german version and includes a lot of useful hints. After 2 therapies with cladribine ( first 10 years ago, 2nd 5 years ago) i took some infusions with CD20 rituximab last year ( first one in clinic in combination with cladribine). After the first infusion caused a heavy reaction , the following threatments caused less heavy reactions in combination with additional drugs and 100 mg cortison. In summary, It seems to be very successful with no more hairy cells in blood and in the backbone and a normal blood status without additional drugs.( i reached now best blood values since the last ten years) Although theres a high risk of infections during the rituximab threatments because the whole building of white blood cells is suppressed, even 3 or 4 month after finishing the last threatment. Patients should know that a simple herpes virus can affect a serious desease in this time. A starterpack of filgastrim injections i.p. each 3rd day is recommended to give some protection until normal production of white blood cells in spine starts again. Maybe this is helpful for some other patients. Michael B. 20.08.2012 — Preceding unsigned comment added by 87.162.191.88 (talk) 20:19, 20 August 2012 (UTC) 93.204.45.23 (talk) 17:01, 12 May 2017 (UTC)Additional message to my post from 20.08.2012 : Its now 6 years ago after the last threatment as described in 2012 . Ive got normal blood status with healthy values . Allergic reactions increased e.g. against plants or grass with red eyes and a running nose, but i can handle it. Blood status is controlled each 6 month, with no failures.As described in the article , the main indicator thing when having HCL is to control the amount of thrombocytes. in my case it is between 150000 - 200000. You will never reach the former values of thrombocytes ( before getting HCL) but it is nessecary to keeep it at least over 100.000- thats what my doctor says. Michael B. 12.05.2017[reply]


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I dont agree that fludarabine is ineffective againts hairy cell leukaemia. Fludarabine is porbably as effective as 2CDA, but has less studies.

see for instance http://content.nejm.org/cgi/content/extract/330/10/691


Well... actually, no. Fludarabine is actually better studied and used more, in general, than either cladribine or pentostatin -- it's just not used, nor particularly effective, for HCL. Here's why I think the statement is justified as it stands:
  • Fludarabine is not approved for use against HCL by any government regulatory agency (e.g., the FDA in the U.S. or any of the European agencies)
  • The folks at Kreitman's lab at the NIH (home of the BL22, HA22, and LMB-2 clinical trials) say that it basically has no value for HCLers.
  • Nobody, including Saven [who wrote the article you cite] actually uses it to treat HCL. (Not being able to read the whole article online, I can't tell you what he says about it, just that he mentions its existence in that article.)
  • Fludarabine (in the U.S.) has got a big black-box warning because it's very messily fatal when combined with pentostatin.
  • It appears that two HCLers are dead from an extremely rare autoimmune disease after fludarabine treatment: http://www.blackwell-synergy.com/links/doi/10.1046/j.1365-2141.2000.02058.x/abs/
http://www.meb.uni-bonn.de/cgi-bin/mycite?ExtRef=MEDL/91121422 is the first report of fludarabine in treating HCL. They got two partial responses and one minor response -- which is pretty much a failure, compared to the numbers that you get from cladribine and pentostatin (80% complete responses, 15% partial responses), even when you consider that the patients in this fludarabine study weren't treatment-naive.
But if that's your definition of "effective," then perhaps we just have different standards for what constitutes effective. It seems to me that cladribine and pento are better for HCL. (I freely admit that fludarabine is better for regular B-CLL.)
To be fair, fludarabine may be slightly more neglected in HCL than it needs to be; there is a possibility that it could be at least partly helpful to some patients. However, by the time you're resistant to cladribine and pentostatin, then you're caught in the cross-resistance problem involving this class of drugs.[1] And no one starts with fludarabine -- why would anyone want to start with the drug that's least likely to work for them? WhatamIdoing 06:24, 5 March 2007 (UTC)[reply]
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Hi, I've added a link in the external links section to a patient-focused page on HCL... HenryScow (talk) 12:00, 15 June 2011 (UTC)[reply]

Guideline

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Classic variant doi:10.1182/blood-2016-01-689422 JFW | T@lk 20:46, 2 February 2017 (UTC)[reply]

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