what is delta spd

HPS

What is Delta SPD?

Delta storage pool deficiency is a bleeding disorder caused by a deficiency in dense granules in the platelets. Dense granules release chemicals in the clotting process and help platelets stick together to form clots. Delta SPD was discovered as a unique illness in the 1970s. Patients who had it prior to that time may have been diagnosed with a more general condition, such as "coagulopathy."

Delta SPD can vary in severity based on gender, degree of dense granule deficiency, and other factors. Find information about symptoms, diagnosis, and treatment options below.

Symptoms

The symptoms of Delta SPD are similar to other bleeding disorders. It causes bleeding in the mucous membranes (nose, mouth, etc.), bruising, and potentially severe bleeding following injuries and surgery. Women with Delta SPD may experience excessive menstrual bleeding and bleeding complications from pregnancy and childbirth.

For more information about symptoms, visit the Canadian Hemophilia Society website about symptoms of platelet function disorders.

Diagnosis

Diagnosis of Delta SPD comes after a careful history and physical exam and bloodwork including:

• clotting time (which may or may not be normal)
• platelet aggregation tests (which often show an abnormality in response to epinephrine)
• electron microscope study of the platelets (which will show abnormally low numbers of dense granules per platelet).

The electron microscope study is perhaps the most specific diagnostic tool, as it can differentiate delta storage pool deficiency from other platelet disorders. Platelets normally have an average of 4-6 dense granules per platelet. Patients with delta storage pool deficiency may have 3, 2, or even lower numbers on average. Patients who have no dense granules may have Hermansky-Pudlak Syndrome.

For more information about diagnosis, please visit the Canadian Hemophilia Society website about diagnosing a platelet function disorder.

Treatment Options

The treatment for Delta SPD is generally to relieve symptoms, as there is no medicinal way to increase the number of dense granules in one's platelets. Two medicines can help alleviate symptoms. They are:

• DDAVP -- This medicine is a synthetic version of a hormone called vasopressin, which stimulates release of von Willebrand's factor and helps improve blood clotting. It comes in three forms: nasal spray, pills, and injections.
• Amicar -- This medicine interrupts the breakdown of clots and allows clots some extra time to stop bleeding in patients with bleeding disorders. It comes in pill form and can be taking at a variety of doses and time schedules.

Patients who are experiencing a life-threatening bleed or surgery may require a platelet transfusion to effectively manage their condition. Though Delta SPD patients are not deficient in platelets, the addition of normal platelets (with adequate numbers of dense granules) helps the blood clot more normally until the patient is out of danger.

Patients with any type of bleeding disorder are generally advised to avoid aspirin and NSAIDS (ibuprofen, naproxen, etc.). In addition, patients may decide to avoid activities that put them at risk for bleeding. Women, in particular, may want to carefully discuss pregnancy and childbirth with their medical team and their family, in order to address the risks and potential complications in their case.

For more information, please visit the Canadian Hemophilia Society websites for treating a platelet function disorder and precautions to take regarding over the counter medicines, foods, and activities.

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This information was compiled from a number of sources. The Canadian Hemophilia Society website about delta SPD has great (and more complete) information about all of these issues. Please consult your doctor for diagnosis and treatment.

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