Wet mucocutaneous bleeding (Oral hemorrhagic bullae)

Specialty: Hematology.

• Wet purple
• introoral hemorrhagic bullae
• active mucosal hemorrhagic diathesis

Why it occurs

• Severe primary immune thrombocytopenia / ITP (accelerated peripheral destruction of platelets mediated by IgG type autoantibodies directed against platelet membrane glycoproteins GPIIb/IIIa, with platelet numbers critically lower than 10,000/μL that compromise primary hemostasis of the oral mucosal territory)
• Thrombotic thrombocytopenic purpura / TTP (severe deficiency of the metalloprotease ADAMTS13 with diffuse microvascular formation of platelet-rich thrombi that generates consumption thrombocytopenia and multiorgan ischemia)
• Severe medullary aplasia (deep pancytopenia with no platelet production in the hematopoietic niche)
• Disseminated intravascular coagulation / DIC (massive consumption of platelets and coagulation factors secondary to sepsis, polytrauma or advanced neoplasms)
• Bernard-Soulier syndrome or severe decompensated Glanzmann's thrombasthenia.

Initial workup

Immediate complete blood count with automated count and urgent peripheral blood smear (essential to verify the real number of platelets, rule out pseudothrombocytopenia due to EDTA using a smear with sodium citrate and actively look for the presence of schistocytes, the presence of which critically guides the diagnosis of thrombotic microangiopathy such as TTP); complete coagulation profile (prothrombin time, activated partial thromboplastin time, decreased fibrinogen and very elevated fibrinogen/D-Dimer degradation products in case of suspected DIC); bone marrow aspiration and biopsy (to evaluate the presence and number of megakaryocytes, increased in ITP and absent in marrow aplasia); determination of ADAMTS13 activity (confirmatory of PTT if <10%).

Standard management

• Intravenous human immunoglobulin / IVIG — administered at a dose of 1 g/kg body weight per day for 1 or 2 consecutive days; acts by competitively blocking the Fc receptors of splenic macrophages, achieving a rapid and defensive increase in the number of platelets in 24-48 hours in cases of severe ITP with wet purpura
• Methylprednisolone — high-potency intravenous systemic glucocorticoid to stop platelet immune destruction and stabilize vascular capillary fragility
• Romiplostim or Eltrombopag — thrombopoietin receptor agonists that stimulate megakaryocytic production in bone marrow in patients with refractory ITP
• Rituximab — anti-CD20 monoclonal antibody indicated in persistent ITP or refractory TTP to eliminate autoantibody-producing lymphocyte clones
• Platelet transfusion (indication of extreme urgency only if there is immediate life-threatening bleeding, given that in ITP or PTT the transfused platelets are destroyed or consumed almost instantly and in PTT they can exacerbate the microthrombotic phenomenon).