Severe Hemophilia A: Characteristic Clinical Findings
The most characteristic finding in severe Hemophilia A is (A) large subcutaneous hematomas and recurrent hemarthroses (joint bleeding), which occur spontaneously without trauma in patients with factor VIII levels <1 IU/dL. 1, 2
Why Option A is Correct
Severe hemophilia A is defined by spontaneous bleeding into joints (hemarthrosis) and muscles (hematomas) as the most frequent clinical manifestations. 1, 2 This distinguishes it fundamentally from platelet disorders:
- Patients with factor VIII activity <1 IU/dL experience spontaneous bleeding without the need for trauma or injury as a trigger 2
- Joint bleeding (hemarthrosis) is the most frequent clinical manifestation of severe hemophilia, occurring recurrently and leading to hemophilic arthropathy if not appropriately managed 3, 4
- Muscle bleeds and large subcutaneous hematomas are characteristic deep tissue bleeding patterns seen in coagulation factor deficiencies 1
- These bleeding episodes typically appear early in life, with joint and muscular bleeding evident in early childhood 2
Why the Other Options are Incorrect
Option B (petechiae and purpura) is characteristic of platelet disorders or vascular defects, NOT hemophilia A:
- Petechiae and purpura represent superficial bleeding from capillaries and small vessels 1
- These findings occur in thrombocytopenia, platelet dysfunction, or vasculitis—not coagulation factor deficiencies 1
- Normal platelet count and bleeding time rule out platelet disorders in patients with hemophilia A 5
Option C (poor wound healing and re-bleeding) is not the primary or most characteristic feature:
- While patients may experience delayed bleeding after trauma or surgery, this is more typical of mild hemophilia 1, 2
- The hallmark of severe disease is spontaneous bleeding, not wound-related issues 1
Option D (thrombosis) is actually a complication of treatment, not the disease itself:
- Thrombosis can occur as an adverse effect of bypassing agents (recombinant FVIIa, activated prothrombin complex concentrates) used to treat hemophilia 1
- Prothrombin complex concentrate use may increase thrombosis risk and is contraindicated in standard hemophilia management 1, 6
- Hemophilia is fundamentally a bleeding disorder, not a thrombotic condition 1
Clinical Significance
The pattern of deep tissue bleeding (joints and muscles) versus superficial bleeding (petechiae) is the key clinical distinction:
- This bleeding pattern directly results from the inability to form stable fibrin clots due to factor VIII deficiency 1
- Recurrent hemarthroses lead to progressive hemophilic arthropathy, characterized by joint remodeling, chronic pain, and reduced quality of life 3, 4
- Recognition of this pattern is essential for diagnosis and distinguishing hemophilia from platelet disorders or other coagulopathies 1, 5