Henoch-Schonlein Purpura with thrombocythaemia: an abnormality in Smad4 expression?
We discuss a patient with Henoch-Schonlein Purpura (HSP) and a thrombocythaemia which was diagnosed as a coincidental Essential Thrombocythaemia. We suggest that deficiencies in Smad4 expression may allow for escape thrombocythaemia under the influence of the high levels of TGF-beta found in HSP. With normal Smad4 expression TGF-beta provides inhibition of thrombocyte proliferation. While this needs further elucidation, it could lead to a new approach to classification and management of HSP.
Brostoff
Joshua M
JM
Central Middlesex Hospital, London, UK. joshbrostoff@doctors.org.uk
Ranganna
Gopinath M
GM
Colaco
C Bernard
CB
D001706:Biopsy; D001792:Blood Platelets / Q000502:physiology; D049109:Cell Proliferation / Q000187:drug effects; D062109:Dermatologic Surgical Procedures; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D011695:IgA Vasculitis / Q000175:diagnosis* / Q000188:drug therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D010976:Platelet Count; D011239:Prednisolone / Q000627:therapeutic use; D012867:Skin / Q000098:blood supply; D051901:Smad4 Protein / Q000378:metabolism*; D013920:Thrombocythemia, Essential / Q000175:diagnosis*; D013997:Time Factors; D016212:Transforming Growth Factor beta / Q000378:metabolism / Q000494:pharmacology; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D051901:Smad4 Protein; D016212:Transforming Growth Factor beta; D011239:Prednisolone; D009173:Mycophenolic Acid