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Haarzell-Leukämie-Hilfe e.V. |
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Hairy Cell Leukemia - Variant (HCL-V)
This is my case history for those who have shown interest in HCL-V. HCL is very rare (600 cases a year in US), while HCL-V is rarer again.
HCL Treatments The medical literature available on the net on HCL is quite numerous and provides evidence-based knowledge on the latest treatments. For example a trial undertaken at the Royal Marsden Hospital, London, compared two purine analogues, DCF and 2-Cda, on HCL patients. In one group 159 patients with HCL (8 with HCL-V) were given DCF. While the results were good for HCL suffers, only 4 of the 8 HCL-V suffers responded to DCF and none attained CR. Another group of 26 patients (two with HCL-V) were treated with 2-Cda. Again, the HCL patients reacted well, while of the 2 HCL-V patients, one did not respond at all and both died of active disease. The rest of the medical literature available on the net on HCL-V is equally depressing and supports the above study conclusions that DCF and 2-Cda are ineffective in the treatment of HCL-V. There was one patient in Italy who obtained CR when his spleen was radiated. I raised this with my Oncologist who strongly recommended against radiation because it causes very large lesions, which make it almost impossible to remove the spleen afterwards, if that becomes necessary. Further if radiation of the spleen worked then a splenectomy (complete removal of the spleen) may work. The conclusion from the medical literature search on the net is that there are no modern treatments for HCL-V (N.B. this situation has changed since this was written last year - monoclonal antibody which kill CD20 antigens have been tested successfully on HCL - HCL-V also has the CD20 antigen!) My History The diagnose of HCL-V was, like most leukaemia, more by accident than good medical management. About a year before being diagnosed I had notices slow weight loss (pants a bit lose around the waist). There were two medical events associated with the weight lose. Firstly, having been treated for 30 years for hypertension with a-blockers and ACE drugs, the blood pressure fell markedly so I could stop all blood pressure medication (proves conclusively that weight has a lot to do with hypertension). Secondly, I started to pass uric acid gravel in the urine. A blood test and IVP test (injecting dye and X-raying the kidneys and bladder) did not show stones or uric acid in the blood. The same test repeated a year later for another reason, after being diagnosed with HCL-V, reported an enlarged spleen. The original X-ray also showed an enlarged spleen but it was not reported. By Christmas 1997, I became more interested in my weight and started to record it. I was 86 Kg at that stage. My wife and I went on an intensive 3 month holiday by organised bus tours through Europe, UK, Ireland and Israel in April 1998. If you have ever been on these types of bus tours, you know how strenuous they are - in and out of the bus, walking miles and changing hotels every day. There was no indication of any problem. We returned to Australia at the end of July. I had lost another 3 Kg but it was not unexpected. In September 1998, I got flue like symptoms, a cough that did not go away and excessive night sweating (totally drenched). The doctor was suddenly very interested in the continued weight loss and night sweating. He went ballistic when he did a physical examination - for the first time, I might add - and found the spleen grossly enlarged. An ultra-sound confined the grossly enlarged spleen and associated lymph nodes, which suggested leukaemia. The oncologist had blood tests and bone marrow biopsy done immediately at the local hospital and HCL-V was diagnosed. He was of the opinion that I had the disease for at least two years. The blood test showed very low Hb (90 g/L compared with normal range of 135 - 180 g/L) (Do you feel tired? No), low platelet count 50 x 109 /L compered to 150 -400 (Do you bruise easily or your gums bleed? No.) and very high WBC (Lymph) of 28.3 x 109 /L compared to 0.8 - 4.0. In addition, I was found to have an under active thyroid. The oncologist was of the opinion that the disease was largely centred on the spleen and the bone marrow was relative free of hairy cells. He recommended traditional leukaemia oral chemo to see if the spleen could be reduced. I had three courses of chemo:
The spleen progressively reduced after every treatment but I had bouts of secondary infections, which required hospitalisation and muscle wastage from arms and legs from the prednisolone. I also developed prednisolone-induced Type 2 diabetes, which has been confirmed by a gluose tolerance test. The diabetes is being treated with diet and exercise (walking 30 minutes a day and golf twice a week). In December 1998, the oncologist gave me three options:
Since having the spleen removed, I have regained my original weight (now 90 Kg), got some hypertension back and some mild pain in the joint and muscles. The muscle tone is slowly returning with exercise. I feel great but the blood tests still shows some activity. A table of the important "numbers" are given below. Peripheral Blood Count
* two weeks after upper respiratory viral infection ** 24 hrs later after a round of golf!! zurück zur Übersicht der Berichte | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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