Some of the common types of non-immune-mediated platelet destruction include:
Disseminated Intravascular Coagulopathy (DIC)
DIC is the abnormal formation of small blood clots within the blood vessels. This process consumes clotting factors and platelets resulting in a reduced platelet count and increased bleeding. DIC can result from a variety of infections, traumas, diseases, and some drugs.
Website Resources:
Thrombotic Thrombocytopenic Purpura (TTP) / Hemolytic Uremic Syndrome (HUS)
These related diseases are both characterized by low platelet counts, fragmentation and destruction of red blood cells and organ damage, especially the kidneys. TTP can be caused by the inhibition or an inherited defect of the von Willebrand Factor cleaving enzyme, ADAMTS13 and some drugs. HUS is seen more frequently in children after a bacterial infection.
Organizations:
- TTP Network
- www.AnsweringTTP.org
- https://www.nhlbi.nih.gov/health/thrombotic-thrombocytopenic-purpura
- https://rarediseases.org/rare-diseases/thrombotic-thrombocytopenic-purpura/
- https://www.ncbi.nlm.nih.gov/books/NBK556038/
- https://rarediseases.org/rare-diseases/atypical-hemolytic-uremic-syndrome/
- https://www.ahuscanada.org/
Enlarged Spleen (splenomegaly)
The spleen usually stores about one-third of the body’s platelets. If it becomes enlarged due to various diseases, the spleen will store additional platelets leading to thrombocytopenia. In ITP, the spleen is expected to be a normal size, not significantly enlarged. An enlarged spleen may be a sign of a secondary thrombocytopenia and should be a trigger for additional investigations.
Website Resources:
- https://rarediseases.info.nih.gov/diseases/7684/splenomegaly
- https://www.osmosis.org/answers/splenomegaly
Henoch-Schönlein Purpura (HSP)
Henoch Schönlein Purpura (HSP) is a type of vasculitis, which means inflammation of the blood vessels. It does not cause platelet destruction, and symptoms are not due to a low platelet count although they may resemble ITP symptoms. The classic symptom of HSP is a red to dark purple rash, called “purpura”, which is often most severe on the legs and buttocks. Other symptoms include painful swelling around the joints and abdominal pain. Many children with HSP recover completely, but up to one-third of patients can develop kidney disease. Treatment depends on the individual child’s symptoms. Monitoring for kidney disease plays an important role in early detection and treatment if this complication happens.
In North America, HSP is the most common form of vasculitis in children. It is most common in ages 3-15 years and is more common in boys than girls. It rarely occurs in adults. HSP can occur at any time of the year, though it is more common in the winter.
Resources:
- https://rarediseases.org/rare-diseases/henoch-schonlein-purpura/
- https://www.hopkinsvasculitis.org/types-vasculitis/henochschnlein-purpura/
- https://rarediseases.info.nih.gov/diseases/8204/henoch-schonlein-purpura
Pseudothrombocytopenia
Pseudothrombocytopenia is a misdiagnosis of thrombocytopenia due to platelet clumping caused by a reaction to chemicals in the laboratory sampling collection process, rather than by a disease affecting platelet production or destruction. The clumping causes the automatic counters to undercount platelets which is why most places will not run the test if there is clotting in the tube. This is a lab error and not a representation of an underlying platelet disorder.
Pseudothrombocytopenia is a misdiagnosis thrombocytopenia due to platelet clumping caused by a reaction to chemicals in the laboratory sampling collection process, rather than by a disease affecting platelet production or destruction. The clumping causes the automatic counters to undercount platelets which is why most places will not run the test if there is clotting in the tube. This is a lab error and not a representation of an underlying platelet disorder.
