Welcome, Guest. Please Login
MCD - Mast Cell Disorders
  Signup for free on our forum and benefit from new features!
  HomeHelpSearchLogin  
 
Page Index Toggle Pages: 1
Send Topic Print
Indolent systemic mastocytosis without skin involvement vs. isolated bone marrow mastocytosis (Read 7733 times)
Riverwn
FORUM MODERATOR/ADVISOR
*******
Offline



Posts: 993
Gainesville, Florida
Indolent systemic mastocytosis without skin involvement vs. isolated bone marrow mastocytosis
06/22/11 at 19:13:49
 
Indolent systemic mastocytosis without skin involvement vs. isolated bone marrow mastocytosis

Luis Escribano,1 Ivan Alvarez-Twose,1 Andres Garcia-Montero,2 Laura Sanchez-Muñoz,1 Maria Jara-Acevedo,2 and Alberto Orfao2
1 Instituto de Mastocitosis de Castilla La Mancha, Hospital Virgen del Valle, Toledo
2 Servicio General de Citometría, Centro de Investigación del Cáncer (IBMCC-CSIC/USAL) and Departamento of Medicine, University of Salamanca, Spain. Spanish Network on Mastocytosis
Correspondence: Luis Escribano, MD, PhD, Instituto de Mastocitosis de Castilla La Mancha, Hospital Virgen del Valle, Toledo, Spain; E-mail: lescribanom@sescam.jccm.es
Keywords: bone marrow mastocytosis, fracture, osteoporosis

   *  Other Sections▼
         o References


We have read with great interest the paper by Zanotti et al.1 about the description of “Isolated bone marrow mastocytosis” (BMM) as an underestimated subvariant of indolent systemic mastocytosis (ISM). This is a very interesting paper in which an in depth study was performed in a total of 99 consecutive ISM patients, diagnosed either prospectively or retrospectively according to the 2008 WHO classification.2 However, the term used to define the series of BMM might not be the most appropriate and may lead to confusion in the field.
In Zanotti’s report, after an in depth study, 46 of 84 patients (54.7%) who were referred because of unexplained/recurrent anaphylaxis or severe allergic reactions to hymenoptera sting in the absence of typical skin lesions (e.g. mastocytosis in the skin; MIS), were classified as BMM.
It is noteworthy that, indolent systemic mastocytosis (ISM) in the absence of skin involvement (ISMs−) has been previously recognized and described by several groups. In fact, it was first recognized as a clinical entity in 19913 and later, its clinical, biological, morphological, immunophenotypical and KIT mutational characteristics have been established in a large series of cases.4 In addition, Dr. Zanotti co-authored an elegant paper on hymenoptera venom anaphylaxis and mastocytosis in which patients lacking MIS but fulfilling criteria for SM are classified as ISMs−.5 Detailed analysis of the reported isolated BM mastocytosis cases1 shows that most likely they correspond to the previously identified ISMs-patients.
It should be emphasized that ISMs− clearly differs from isolated BM mastocytosis in that the latter is an exceptional subcategory of ISM in the absence of both skin lesions and MC-mediator related symptoms. In addition, subjects with isolated BM mastocytosis typically show a low MC burden2;6 and their diagnosis is occasionally made during a BM study for pathological conditions other than mastocytosis, due to its clinically silent behavior (only 5 cases in our data base, Spanish Network on Mastocytosis-REMA−, data not shown).
Conversely, ISMs− is a “true” systemic disease based on the observation of systemic mast cell-mediator related symptoms, difficult to explain in a BM restricted MC condition. ISMs− is also frequently associated with osteoporosis and presence of multilineal KIT mutations is detected in 3 out of 47 cases (6.7%) in our published series4 and in a total of 4 out of 62 (6.4%) cases in the updated REMA’s data base (REMA, unpublished data, 2011) which confers to these patients an increased probability for disease progression.7
The term isolated BM mastocytosis may lead the readership not familiar with this disease to consider this condition to be benign and of a localized nature, when in fact it may be associated with life-threatening or even fatal anaphylaxis with possibly around 5% of cases progressing to aggressive disease.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3069249/
Back to top
 

~~~Count  Your Blessings!~~~
 
IP Logged
 
rickypoarch
Rookie
*
Offline


I Love YaBB 2!
Posts: 4

Re: Indolent systemic mastocytosis without skin involvement vs. isolated bone marrow mastocytosis
Reply #1 - 03/13/12 at 17:50:18
 
IS HIS THE SAME HAS THE SM-AHNMD ?,  I HAVE BEEN LOOKING FOR SOMEONE HOW HAS THIS BUT I CAN NOT FINE ANYONE WITH THE SAME THING.
Back to top
 
 
IP Logged
 
Anaphylaxing
Guru
*****
Offline


Hang in there! You can
do it!
Posts: 836

Re: Indolent systemic mastocytosis without skin involvement vs. isolated bone marrow mastocytosis
Reply #2 - 03/14/12 at 14:41:52
 
I wonder what the serum tryptase was in these patients
Back to top
 
 
IP Logged
 
peter
Mentor
****
Offline


I Love YaBB 2!
Posts: 294

Re: Indolent systemic mastocytosis without skin involvement vs. isolated bone marrow mastocytosis
Reply #3 - 03/25/12 at 11:45:37
 
I know i have carried this all my life and it was activated by the wasp and venom injections. I don't have skin involvement. I try my best to communicate but don't do as well as I would like. Here is my experience:-

At 16 years old, with a history of anaphylaxis to bees with severe full body swelling.

14-3-2007 I suffered catastrophic anaphylaxis to a wasp sting with a tryptase of 200. After this I was having mild anaphylaxis like symptoms daily feeling like I was going to pass out. One doctor said it was was post traumatic stress (PTSD). I thought I was going to have a nervous breakdown.

8-6-2007 with advice from doctors I started mixed venom desensitization injections. This caused anaphylaxis reactions, despite taking antihistamines 30 minutes before the injections.

10-5-2007 Tryptase at 41.7, doctors were advised of Systemic Mastocytosis but no one told me that diagnosis.

As the reactions became more severe and more frequent, the symptoms affected my ability to function properly. I insisted on having a cat scan of my brain as I thought I must have had brain damage. I also had different body pains so I had an MRI and Xrays which I paid for myself.

The reactions and pains continued and were getting worse after [41 injections] and 16 months.

19-9-2008 I stopped the desensitization injections. The doctor would not refer me to a specialist.

The reactions continued on a daily basis and I began to have weird pains in all parts of my body, such as severe gut pain, which put me in hospital for one day. Insomnia and night sweating, gout,
sciatica, and on several occasions I went to doctors and the hospital with anaphylaxis symptoms

29-9-2009 I was diagnosed with SM Kit 816v. Cd117++. Cd25. Cd33.cd34+ with blood tryptase of 32

By this time I was having severe pain at times and continuous bad
pain 24 hr per day 7 days a week with more severe reactions for no reason 6/8/10 times a day.

I went from 90kg to 69kg (198 lbs. to 152 lbs.) I went to hospital with bowel obstruction and inflamed bowel, gastrointestinal tract bleeding. My toe nails, finger nails and teeth went white and brittle over 4 months. 5 teeth broke off bit by bit back to the gums. Full body osteoarthritis more unusual symptoms.

This is where I am currently:-
nerve pains / migraine headaches / muscle pain / tendon tightening / severe bone pain and flushing

Cognitive impairment makes me not able to think straight, especially when trying to do any task. Also extreme unrelenting fatigue.

1-2-2010 bone density z-score of -2.8 and
12-1-2011 bone density z score -3.8 osteoprosis

31-3-2010 tryptase 46.8 and
6-5-2010 tryptase 62.1.and
19-1-2011 tryptase 50 .

15-10-2009 bone marrow biopsy showed 4 aggregates of scattered spindle shaped mast cells

I was given the Patient information sheet for the trial of chemotherapy called PKC412 Midostaurin which is a current clinical trial ongoing worldwide.

17-8-2010 bone marrow biopsy showed small clusters of spindle shaped mast cells

First diagnosis was MCAS-SM second diagnosis IA-SM - Now BMM with progression WHO knows???

1-11-2011 Medications:-

for Osteoprosis (T score -3.7) is zometa infusion caltrate 600mg taking 2 per day;

Nalcrom oral cromoglycate 200mg 4 times a day

Loratadine 10mg 2 or 3 per day

Ranitidine 300mg 2 times per day

Phenergan 25mg 1 a night

My comments and final thoughts on my experience are this...

I experienced severe mast cell activation with continuous reactivation of mast cell degranulation caused by desensitization injections.

Continual progression in SM/ Bone marrow mastocytosis

All dates and information confirmed by medical records

march 2012

the desensitization was geven to me by a GP  HE could the reactions
post trumatic strss and over dosed me this coused UN CONTROLED HYPOSENSERTIV IMMUNE SYSTEM
there have been BIG CHANGERS in VIT now this wont happen to anyone
peter

Back to top
 
 
IP Logged
 
Page Index Toggle Pages: 1
Send Topic Print