Most ITP is self-limiting and not life-threatening. Spontaneous intracranial haemorrhage is the most feared complication but occurs in 20,000–30,000/µL) rather than normalise the count. Many patients with chronic ITP live normal lives with a count of 30,000–80,000/µL without treatment.

🩹 Men's Health

ITP & Bleeding Disorders (Nigeria Guide)

Q: Is haemophilia only in boys?

Haemophilia A and B are X-linked recessive — almost exclusively in males. Females are carriers; some carrier females have borderline factor levels and mild bleeding symptoms. Von Willebrand disease (the most common inherited bleeding disorder) affects males and females equally. Women with VWD often present with heavy periods, while men present more with surgical or trauma bleeding.

Immune thrombocytopenia, haemophilia, and von Willebrand disease — diagnosis and care in Nigeria

Bleeding disorders — conditions causing excessive or abnormal bleeding — range from the common (immune thrombocytopenic purpura — ITP, von Willebrand disease) to the rare (haemophilia). In Nigeria, haemophilia and other inherited bleeding disorders are significantly underdiagnosed. Many patients are labelled as 'clotting badly' or undergo unnecessary procedures without a proper diagnosis. The Haemophilia Foundation of Nigeria and its chapter centres provide specialist care and factor concentrate access.

Signs and Symptoms

✓ Petechiae — tiny pinpoint red spots under the skin from platelet problems

✓ Bruising easily or spontaneously — out of proportion to the injury

✓ Prolonged bleeding after cuts, dental extractions, or minor procedures

✓ Heavy menstrual bleeding (menorrhagia) — often the presenting symptom of von Willebrand disease in women

✓ Haemarthrosis — bleeding into joints (haemophilia) — warm, swollen, painful joints

✓ Muscle haematomas — spontaneous bleeding into muscles

✓ Nosebleeds lasting >10 minutes or recurrent

✓ Blood in urine (haematuria) or stools in severe platelet disorders

Risk Factors

ITP: often follows viral infection, autoimmune condition, or HIV in adults
Haemophilia A and B: X-linked — affects males; females are carriers
Von Willebrand disease: autosomal — affects both sexes equally
Liver disease: impaired clotting factor synthesis
Vitamin K deficiency: fat malabsorption, antibiotic use, neonatal period
Antiplatelet drugs: aspirin, clopidogrel
Anticoagulants: warfarin, heparin

When to Seek Help

EmergencyIntracranial bleeding — sudden severe headache with low platelet count or haemophilia — emergency imaging and factor replacement

EmergencyHaematoma rapidly expanding in the neck or airway area — can obstruct breathing

UrgentPlatelet count <20,000/µL with active bleeding — ITP or other severe thrombocytopenia

SoonUnexplained easy bruising, petechiae, or prolonged bleeding — haematology assessment

Before surgery/dental workKnown bleeding disorder — inform the surgical team well in advance for factor replacement planning

Tests & Diagnosis

🧪 Full blood count with platelet count

Thrombocytopenia (<150,000/µL) detected on FBC. Peripheral smear to exclude pseudothrombocytopenia (platelet clumping)

🧪 Coagulation screen: PT, APTT, fibrinogen

PT prolonged = extrinsic pathway (vitamin K, liver). APTT prolonged = intrinsic pathway (haemophilia A, B, von Willebrand). Both prolonged = disseminated intravascular coagulation (DIC) or severe liver disease

🧪 APTT mixing study

Distinguishes factor deficiency (corrects with normal plasma) from inhibitor (does not correct)

🧪 Factor VIII and IX assay

Confirms haemophilia A (factor VIII deficiency) and B (factor IX deficiency). Levels 1–5% = severe, 5–40% = moderate

🧪 Von Willebrand antigen and activity (ristocetin cofactor)

Confirms VWD — the most common inherited bleeding disorder

🧪 Bone marrow biopsy

For suspected aplastic anaemia or thrombocytopenia of unclear cause

Treatment Options

ITP (immune thrombocytopenic purpura) — first-line

Prednisolone 1 mg/kg/day for 2–4 weeks. IV immunoglobulin (IVIG) 1 g/kg for 1–2 days — rapid platelet rise within 24–48 hours; used for severe bleeding or pre-surgery. Dexamethasone 40 mg daily for 4 days — alternative to prednisolone

ITP — second-line (chronic/refractory)

Rituximab (anti-CD20) — B-cell depletion; available at LUTH and UCH. Thrombopoietin receptor agonists (eltrombopag, romiplostim). Splenectomy — removes the site of platelet destruction and anti-platelet antibody production; very effective but risk of post-splenectomy infection

Haemophilia A — factor VIII replacement

Recombinant factor VIII concentrate (Advate, Xyntha) — on-demand for bleeds. Prophylaxis — regular factor infusions to prevent bleeds in severe haemophilia. Factor concentrate available through the Haemophilia Foundation of Nigeria (HFN) and Federal Ministry of Health. Desmopressin (DDAVP) for mild haemophilia A — stimulates release of stored factor VIII

Haemophilia B — factor IX replacement

Factor IX concentrate. Extended half-life products (Alprolix, Idelvion) require less frequent infusions — twice weekly or weekly

Von Willebrand disease

Desmopressin (DDAVP) for type 1 VWD — releases stored VWF and factor VIII. Tranexamic acid for mucosal bleeding and menorrhagia. VWF/factor VIII concentrate for severe VWD or types 2 and 3. Combined OCP for menorrhagia in women with VWD

Haemophilia Foundation of Nigeria

HFN chapters provide: free factor concentrate access (limited supply), treatment education, physiotherapy support. Contacts: Lagos (LUTH), Ibadan (UCH), Kano (AKTH), Port Harcourt, Enugu. Register affected patients for access to factor

Frequently Asked Questions

Is haemophilia only in boys?

Haemophilia A and B are X-linked recessive — almost exclusively in males. Females are carriers; some carrier females have borderline factor levels and mild bleeding symptoms. Von Willebrand disease (the most common inherited bleeding disorder) affects males and females equally. Women with VWD often present with heavy periods, while men present more with surgical or trauma bleeding.

Is ITP dangerous?

Most ITP is self-limiting and not life-threatening. Spontaneous intracranial haemorrhage is the most feared complication but occurs in <1% of cases, usually with platelet counts <10,000/µL. The goal of treatment is to maintain a 'safe' platelet count (>20,000–30,000/µL) rather than normalise the count. Many patients with chronic ITP live normal lives with a count of 30,000–80,000/µL without treatment.

Can someone with a bleeding disorder have surgery?

Yes, with specialist planning. Factor levels must be corrected to near-normal before surgery and maintained postoperatively. This requires close coordination between the haematologist and surgical team. Never perform elective surgery in a known bleeding disorder patient without haematology input — the consequences can be fatal.

Where can I get factor concentrate in Nigeria?

The Haemophilia Foundation of Nigeria (HFN) distributes donated factor concentrate to registered patients through partner hospitals. Contact HFN Lagos (+234 803 304 6826) or your nearest teaching hospital haematology department. Factor is sometimes available at LUTH, UCH, ABUTH, and AKTH. Supply is limited — register early and plan procedures in advance.