Question for Acquired VWD folks

Hi all,

If there are any members of the forum who have acquired VWD as a result of a monoclonal protein disorder (MGUS/myeloma, etc.) or another lymphoproliferative disorder, I would be very curious to hear about which VWD type/pattern you acquired.

The medical literature on the subject is kind of sketchy, with lots of literature review articles stating that VWD acquired as a result of these conditions tends to “typically,” “usually,” or “commonly” conform to a type 2 clinical and multimeric pattern (i.e., disproportionately low RCo to Ag ratio and loss of intermediate/large multimers), but there are many other research articles documenting plenty of cases of type 1 occurring as a result of these conditions. My testing has suggested type 1 on the basis of RCo:Ag ratio and multimer evaluation, but I’m quite worried about monoclonal protein disorders possibly being to blame, since I do have a few symptoms that correspond to myeloma (back pain) AND despite being only 31 years old I do have essentially the one known true risk factor for monoclonal disorders, which is excessive history of vaccination (long story, but I’ve had to be vaccinated against rabies several times due to being stuck living in 2 separate apartments that had bats).

Acquired vWD is my middle name. Just spent a week fighting hematologists for correct protocol to follow. I gave them a tailor-made protocol for acquired VWD with inhibitors from USF Hemophilia Treatment Center and the on-call hematologist ignored it and made up his own protocol (which included taking me outpatient to his infusion facility). It took me several days to convince the hospitalist to stop the on-call hematologist from changing my doctor’s orders. My guy was following the HTC protocol. Finally got my APTT down and enough Alphanate VWF/FVIII factor to stop the GI bleed using the HTC protocol. Bear in mind that on-call hematologists outrank your hematologist and, usually, the hospitalist. BUT, the hospitalist CAN say no if he or she has back-up, like the printed protocol from the HTC.
Because of the inhibitors, IVIG is used to knock down the immune system production of antibodies but IGIV only lasts for a few days then the immune system starts cranking out the inhibitors again and the APTT starts shooting up.I got lucky by being armed with a complete work-up from a recognized HTC. Next bleed I go straight to the HTC 70 miles away. There might be 2000 AVWD patients in the world which explains the paucity of literature.
With respect to MGUS…Yes, I have MGUS and an egg-white allergy—there is some correlation. The egg white allergy is severe and from birth. MGUS (monoclonal gammopathy of undetermined significance) is a disorder of Ig (Immunoglobulin) proteins. My AVWD is within the last half-dozen years. I am 75.
I have no multimeres at all and low FVIII. I bleed like type III or Hemophilia A but correct name is "Severe AVWD). My inhibitors throw a party when I get Alphanate infusion. Bleeding only stops with combination of IGIV and Factor given in appropriate doses, like in my HTC protocol.There is no plausible reason for my development of AVWD. I just as well could have gotten lupus, myeloproliferative diseases or any other platelet disorder. I blame it on an errant cosmic ray hitting a random megakaryocyte or B cell causing a mutation. The inhibitors cause the problem, ie. an auto-immune disease.
ALSO----know the pecking order among the doctors. Fighting all of them may injure the one you need. The Hospitalist ranks low but has to check the approval box for all protocols and the specialist can’t easily overrule them, just bully them. It’s kind of like a chess knight-- low in rank but he has a few cool moves if he is backed up.

John the elder***** who needed a break from thinking about AVWD for a while.