Postural orthostatic tachycardia syndrome is associated with platelet storage pool deficiency

Kyla

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http://journals.lww.com/md-journal/...l_orthostatic_tachycardia_syndrome_is.46.aspx
(Open access)


Postural orthostatic tachycardia syndrome is associated with platelet storage pool deficiency
Gunning, William T. III PhD, FMSA; Karabin, Beverly L. PhD; Blomquist, Thomas M. MD, PhD; Grubb, Blair P. MD

Section Editor(s): Marchi., Rita

Abstract: Mechanisms have been postulated to explain postural orthostatic tachycardia syndrome (POTS), however, the etiology of this often debilitating disorder remains unknown. We conducted a retrospective case–control study of 181 POTS patients who exhibited/reported bleeding symptoms for a specific platelet (PL) dysfunction disorder, delta granule storage pool deficiency (δ-SPD).

Patients were included only if results of blood tests for δ-SPD were available. Electron microscopy was utilized to diagnose δ-SPD. An ELISA assay was used to determine serotonin (5HT) concentration in PLs and medical record review was employed to collect patients’ clinical symptoms.

The most common bleeding symptom was easy bruising (71%) but frequent nose bleeds, heavy menstrual bleeding, and a family history of bleeding were also commonly reported. Of the patients studied, 81% were diagnosed with δ-SPD. Our investigation of 5HT concentration extracted from PLs revealed significantly lower levels of 5HT in POTS patients when compared to that of control subjects. Our data suggest that patients with POTS have significant comorbidities including bleeding symptoms and/or family bleeding histories, and have diminished PL 5HT levels supporting the hypothesis that POTS is a low 5HT level disorder. While we describe a significant relationship with POTS and δ-SPD, this finding does not constitute an etiology for POTS.

Our results establish an additional comorbidity frequently seen in POTS that could explain a number of disparate symptoms often affecting the severity of POTS.
 

Justin30

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By 5HT are they refering to the precursor to seratonin?

I mean like 5HTP?

Would be nice to know if we are not getting this from our food amd therefore showcase a malabsorbtion of mito process not working.
 

adreno

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No increased bleeding here. An SSRI seems to help - somewhat. Too much serotonin OTOH makes OI worse (I'm on half the regular dose).
 
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I've gotten low platelets from various supplements and foods so I would guess baseline is in the low end of normal. Spleen ultrasound was normal.

I have little dots of blood under the skin on my upper arms most of the time. (petechia?)
 

bel canto

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A well-respected hematologist recently told me that SSRI's can lead to an acquired bleeding disorder. As a POTS patient, I've never associated a mild bleeding disorder with that condition. Very interesting.
 

bertiedog

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I do well with sumatriptan (Immigran) which constricts the blood vessels in order to control my almost daily migraines and I believe there is a serotonin connection here but not sure what that is without looking it up.

Pam
 

taniaaust1

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oh no, I bet I have this too. I have a history of abnormal bleeding though my blood also clots faster then its supposed to do due to having a double copy of the MTHFR mutation so I seem to have two different conflicting problems with both a bleeding abnormality and a higher clotting abnormality which shows up on clotting tests.

I had terrible nosebleeds as a child to the point a dr for some strange reason decided to take my tonsils out due to the nose bleeds as he thought that would help (strangely that did seem to help some and I stopped getting the almost daily nose bleeds).

Then I haemorraged during the tonsil removal surgery and needed blood transfusions as they couldnt stop me from bleeding from the surgery. (I also get very heavy periods and my daughter gets severe nose bleeds too and my mother almost bled to death after childbirth and needed blood transfusions).
 

taniaaust1

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I just have looked up more info on this

Delta storage pool deficiency is a platelet function disorder caused by a lack of dense granules and the chemicals normally stored inside them. Without these chemicals, platelets are not activated properly and the injured blood vessel does not constrict to help stop bleeding. This type of bleeding problem can be a feature of other inherited conditions (such as Hermansky-Pudlak syndrome and Chediak-Higashi syndrome).

Symptoms
Symptoms of storage pool deficiencies vary from one individual to the next, but they are usually mild to moderate.

People with storage pool deficiencies may experience:

  • Easy bruising
  • Nose bleeds
  • Bleeding from gums
  • Heavy or prolonged menstrual bleeding (menorrhagia) or bleeding after childbirth
  • Abnormal bleeding after surgery, circumcision, or dental work

http://www.wfh.org/en/page.aspx?pid=655