antiphospholipid antibody , itp

Is there anyone out there who has antiphospholipid antibody syndrome , prothrombin gene variant , and itp? My son has all 3. He is currently dealing with the itp more than the others right now . He has a bleed in his abdomen so they have taken him off Coumadin to help heal it. His platelets have dropped though, so the bleed is not healing. He has had prednidone, ivig, rituxan, but none seem to really work on elevating his platelets. They are telling him that his next option is to have his spleen removed . This is so scary. We are at our wits end with all of this . He is 33 years old. 6 years ago they gave him rituxan when he was first diagnosed with itp. It seemed to work because he did not have any problems until last fall. Is there anyone else out there with the same things? He also only has 1 kidney.

Antiphospholipid syndrome increases the risk of blood clots. So does the prothrombin gene mutation.
Splenectomy also increases the risk of blood clots. That does not sound like a good combination.
I hope they can find a better treatment.

Has the Rituxan had enough time to work? Eight weeks is not unusual, and twelve weeks is still possible.

The following article indicates that danazol, dapsone, and cloroquine are options that have had some success:

asheducationbook.hematologylibrary.org/content/2009/1/233.full#ref-42
doi: 10.1182/asheducation-2009.1.233 ASH Education Book January 1, 2009 vol. 2009 no. 1 233-239
Antiphospholipid antibody syndrome
...
Patients with APS-associated thrombocytopenia are treated in a similar manner to patients with ITP since there are limited data suggesting that anticoagulation is an effective therapy for thrombocytopenia in these patients. Treatment options include glucocorticoids, intravenous immune globulin (IVIg), immunosuppressive agents (azathioprine, cyclophosphamide) and off-label use of newer agents such as rituximab. There are individual case reports of successful treatment of thrombocytopenia in patients with APS using danazol, aspirin, dapsone and chloroquine. ...
...
Treatment of Bleeding in Patients with APS

Bleeding is a less frequent complication than thrombosis in patients with APS. Severe thrombocytopenia can result in bleeding; less commonly, patients with APS may have antibodies directed against prothrombin, resulting in increased clearance of prothrombin and low prothrombin levels.45 The site and severity of bleeding will dictate how the bleeding is managed and how urgently this is undertaken. In general, if the bleeding results from antithrombotic therapy, the antithrombotic agent needs to be discontinued, a specific antidote administered (protamine sulfate for heparins, vitamin K for warfarin) and transfusional support given (frozen plasma for heparins or warfarin, prothrombin complex concentrates for warfarin and consideration for red cell transfusions for symptomatic anemia). If the bleeding is associated with thrombocytopenia, or if the patient is taking aspirin, platelet transfusions may be given in addition to treatments that increase the platelet count. Off-label use of recombinant factor VIIa may be considered for severe bleeding unresponsive to other therapies.

Rarely, patients with APS may present with both bleeding and thrombotic complications, and the dominant presentation or most life-threatening complication will dictate how patients are ultimately managed. Patients who are assessed to be at high risk of bleeding should have their anticoagulants withheld or anticoagulants administered using a lower intensity target range while measures to decrease bleeding should be sought. Patients who are assessed to be at high risk of thromboembolic complications despite thrombocytopenia should be anticoagulated and treatments to improve platelet count should be initiated.

I have ITP and have had APS Antibodies. Luckily, I have not yet had a clot. Which antibody does he have? Anticardioipin, Lupus Anticoagulant or anti-β2-glycoprotein-I?

I'd research the splenectomy idea. It doesn't always work for ITP and Rob is right, being asplenic raises the risk of clotting. Could he try Rituxan again since it worked the last time? It can be very difficult to juggle both a bleeding disorder and a clotting disorder. I have seen others go through it. My best to you both.

They are giving him the rituxan again. He has had 3 treatments so far. They were going to only give him 5 total, but have decided to up that to 8. He is also getting the n plate shots weekly. We just hope and pray that the rituxan works again. The last time he only had 4 doses and it kept him good for 6 years . I do not know which antiphospholipid antibody he has . I don't know if we have ever been told actually . He was diagnosed with that and the prothrombin gene variant about 10 years ago. He has had blood clots in his legs. He never had a problem with any of this until about 18 months after he fell from a second story balcony. 18 months
later he developed a blood clot in his leg. He was 19 when he fell.

I'd give Rituxan more time. It's still early. Studies show that two infusions is enough to work for ITP; having eight probably won't do any more than two or four would. I hope he responds soon.

How long has he been using N-Plate? It's not doing anything? N-Plate also raises the clotting risk as you probably know. Hopefully they are being very careful with all of these treatments.

They have been giving him n plate for about a month or 6 wks now.

What are his counts?

He has them checked weekly. When he was admitted to the hospital last week they were 17000. After receiving blood, they dropped to 14000. They gave him ivig and they still did not go up. So they ended up giving him more ivig and rituxan. They went up to 19000. They discharged him at this point. That was on Thursday. He had to go to get an NPlate shot on Friday and will go for his rituxan treatment again this coming Wednesday. They are going to do a bone marrow biopsy on Thursday to see if there is anything going on that they missed. He had a bone marrow biopsy about 6 years ago and it showed nothing at that time.

That is unusual to have an abdominal bleed with counts as "high" as 17k.
Have they located the source?

No. He can't have contrast dye because he only has one kidney. When he was in the hospital a couple of weeks ago they took him off of coumadin and his stomach quit hurting and the bruising went away. After he started his coumadin again , the bruise and the pain returned. They figured that the reason it went away was because by taking him off of coumadin it gave the bleed a chance to heal. So they took him back off. Then his platelets showed low so it is as if the 2 things are working against each other. They said to take him off of the coumadin was the lesser of the 2 evils...meaning they need to get this bleed under control.

Has anyone who has taken rituxan treatments lost there appetite?

According to the Mayo Clinic, loss of appetite is a "less common" side effect of Rituxan, for which you are instructed to check with your doctor or nurse "immediately". (See below). HOWEVER, it is not listed that way in the patient literature, which only instructs you to call your doctor if symptoms such as nausea persist or worsen.

If he has recently stopped taking prednisone, or reduced his dose, that too can cause loss of appetite.

www.mayoclinic.org/drugs-supplements/rituximab-intravenous-route/side-effects/drg-20068057
Check with your doctor or nurse immediately if any of the following side effects occur:

More common

Abdominal or stomach pain
black, tarry stools
bleeding gums
bloating or swelling of the face, arms, hands, lower legs, or feet
blood in the urine or stools
blurred vision
body aches or pain
chest pain
confusion
convulsions
cough or hoarseness
difficulty with breathing
dizziness, faintness, or lightheadedness when getting up suddenly from a lying or sitting position
drowsiness
dry mouth
ear congestion
fever and chills
flushed, dry skin
fruit-like breath odor
headache
hives or welts
increased hunger
increased thirst
increased urination
itching
large, hive-like swelling on the face, eyelids, lips, tongue, throat, hands, legs, feet, or sex organs
lower back or side pain
nausea
nervousness
noisy breathing
pain or tenderness around the eyes and cheekbones
painful or difficult urination
pale skin
pinpoint red spots on the skin
pounding in the ears
shortness of breath
skin rash
slow or fast heartbeat
sneezing
sore throat
sores, ulcers, or white spots in the mouth or on the lips
stuffy or runny nose
sweating
swelling of the tongue or throat
swollen glands
tightness of the chest
tingling of the hands or feet
troubled breathing with exertion
unusual bleeding or bruising
unusual tiredness or weakness
unusual weight gain or loss
vomiting

Less common

Blistering, peeling, or loosening of the skin
blisters in the mouth
blisters on the trunk, scalp, or other areas
burning, crawling, itching, numbness, prickling, “pins and needles”, or tingling feeling
burning, tingling, numbness or pain in the hands, arms, feet, or legs
decreased frequency and amount of urination
diarrhea
difficulty with moving
discouragement
feeling sad or empty
irregular heartbeat
irritability
joint or muscle pain
loss of appetite
loss of interest or pleasure
muscle cramps
muscle pain or stiffness
numbness or tingling in the hands, feet, or lips
pain at the injection site
pain, swelling, or redness in the joints
red skin lesions, often with a purple center
red, itchy lining of the eye
redness of the face, neck, arms, and occasionally, upper chest
stabbing pain
trouble concentrating
trouble sleeping

Incidence not known

Abdominal or stomach cramps
blue-yellow color blindness
blurred vision or other change in vision
burning or stinging of the skin
decreased vision
dilated neck veins
dry cough
extreme fatigue
eye pain, tearing
feeling of discomfort, illness, or weakness
irregular breathing
nosebleed
painful cold sores or blisters on the lips, nose, eyes, or genitals
sensitivity of the eye to light
severe abdominal or stomach pain
severe vomiting, sometimes with blood
sores, welting, or blisters
swelling, stiffness, redness, or warmth around many joints
swollen and inflamed joints
swollen lymph glands
vision loss

I saw that was one of the possible side effects but was wondering if anyone had had any experience with this . Thank you so much for all the info you have been providing .

My son went off food when he was having rituximab. He said it made everything taste metallic and horrible.