How should I initially manage epistaxis in a thalassemia patient receiving chronic transfusions and iron‑chelation therapy?

Initial Management of Epistaxis in Thalassemia Patients on Chronic Transfusions and Iron Chelation

Manage epistaxis in thalassemia patients using standard local hemostatic measures first, while immediately checking platelet count and coagulation parameters, as these patients may have platelet dysfunction from iron overload or thrombocytopenia from hypersplenism, but do not routinely discontinue iron chelation therapy unless severe bleeding persists despite local measures. 1

Immediate Assessment and Local Control

First-Line Hemostatic Measures

• Apply direct pressure by pinching the soft part of the nose for 10-15 minutes while the patient sits upright and leans forward
• Use topical vasoconstrictors (oxymetazoline spray) or apply gauze soaked in 1:1000 epinephrine to the bleeding site
• If bleeding continues, pack the anterior nasal cavity with absorbable hemostatic agents or petroleum jelly gauze 1

Urgent Laboratory Evaluation

• Check complete blood count with platelet count immediately, as thrombocytopenia may be present from hypersplenism or bone marrow suppression 2
• Obtain coagulation studies (PT/INR, aPTT) to exclude coagulopathy from liver dysfunction secondary to iron overload or chronic viral hepatitis 2, 3
• Measure serum ferritin and liver function tests (ALT, AST, albumin) to assess degree of iron overload and hepatic synthetic function 3

Risk Stratification Based on Platelet Count

If Platelet Count <10 × 10⁹/L

• Administer prophylactic platelet transfusion immediately, as this threshold indicates high bleeding risk 2
• Continue local hemostatic measures during platelet transfusion 2

If Platelet Count 10-20 × 10⁹/L with Active Bleeding

• Platelet transfusion is indicated because active epistaxis constitutes a risk factor for bleeding even at this platelet level 2
• The European LeukemiaNet guidelines specify that platelet counts <20 × 10⁹/L with any bleeding risk factor warrant prophylactic transfusion 2

If Platelet Count >20 × 10⁹/L

• Platelet transfusion is generally not required unless bleeding is refractory to local measures 2
• Focus on optimizing local hemostatic control and addressing underlying causes 1

Iron Chelation Management During Epistaxis

Deferiprone-Specific Considerations

• If the patient is on deferiprone, check absolute neutrophil count urgently, as deferiprone carries neutropenia risk that may predispose to mucosal bleeding 3, 4
• Consider temporary switch to deferoxamine if neutropenia (ANC <1500/μL) is present, as this avoids compounding hematologic toxicity 1, 3

General Chelation Approach

• Do not routinely discontinue iron chelation for isolated epistaxis, as maintaining chelation is critical to prevent cardiac iron overload, which accounts for approximately 70% of mortality in thalassemia 1
• Continue deferoxamine or deferasirox unless severe, life-threatening hemorrhage occurs requiring intensive resuscitation 1, 5
• If epistaxis is recurrent or severe, temporarily reduce chelation dose by 25-50% while maintaining some degree of iron removal 5

Addressing Underlying Causes

Hepatic Dysfunction Assessment

• Check albumin and albumin-to-globulin ratio, as reversed A/G ratio indicates cirrhosis from iron overload or chronic hepatitis, which impairs coagulation factor synthesis 3
• Cirrhosis occurs in 10-20% of thalassemia patients and significantly increases bleeding risk 3
• If INR is elevated (>1.5), administer fresh frozen plasma or prothrombin complex concentrate for active bleeding 3

Viral Hepatitis Screening

• Screen for hepatitis B and C if not recently documented, as chronic viral hepatitis affects 4.4-85.4% of transfused thalassemia patients depending on geographic region 2, 3
• Chronic hepatitis accelerates liver fibrosis in the setting of iron overload, compounding coagulopathy risk 2

Mucosal Integrity Considerations

• Iron overload can cause mucosal fragility and telangiectasias that predispose to epistaxis 1
• Optimize iron chelation intensity based on cardiac MRI T2* and liver iron concentration to prevent progressive organ damage 1, 4

Common Pitfalls to Avoid

• Do not assume normal ferritin excludes significant iron overload, as ferritin is falsely elevated by inflammation (particularly in hepatitis C patients) and does not predict cardiac iron loading 1, 3
• Do not discontinue all chelation therapy for minor bleeding, as cardiac iron overload has 50% one-year mortality if untreated and remains the leading cause of death 1
• Do not overlook platelet dysfunction even with normal platelet counts, as iron overload can impair platelet function independent of platelet number 1
• Do not delay hepatitis screening, as chronic viral hepatitis is highly prevalent and accelerates liver disease progression in iron-overloaded patients 2, 3

When to Escalate Care

Indications for ENT Consultation

• Epistaxis lasting >30 minutes despite adequate local measures
• Posterior nasal bleeding requiring posterior packing
• Recurrent epistaxis requiring multiple interventions 1

Indications for Hematology Consultation

• Platelet count <20 × 10⁹/L with active bleeding
• Evidence of coagulopathy (INR >1.5, aPTT >1.5× normal)
• Recurrent epistaxis suggesting underlying platelet dysfunction 2, 1

Transfer to Specialized Thalassemia Center

• If cardiac complications are suspected (chest pain, dyspnea, arrhythmia) in addition to epistaxis, as this constitutes a medical emergency requiring integrated cardiology and hematology expertise 1
• Cardiac iron overload can present with diverse symptoms and has extremely high mortality if not managed at specialized centers 1