General Articles Hematology

Vessel wall abnormalities


Types of vessel wall abnormalities :

1. Hereditary: HHT, EDD, Osteogenesis imperfecta
2. Inflammatory: Bacterial, Viral, Vasculitis.
3. Abn. Collage: Senile, Scurvy, Steroids.
4. Metaboli : Liver dis, Renal, Amyloidosis.
5. Others: Factitious, Paraproteinaemia, Drugs.

Hereditary Hemorrhagic Telangiectasia (HHT)
(Osler – Rendu –Weber synd.)

Clinically :

 A.D., Telengiectasia and small aneurysms on Fingers, lips, Face,
Tongue, Nasal passages, Lungs, G.I.T. / Usually appear at adulthood.
Signif. No. have Pul. A.V. malformation (PAVMS) → Arterial
Hypoxaemia due to Rt. To It. Shunt → paradoxical embolism →
stroke or cerebral abscess.
Recurrent Bleeding e.g. Epistaxis or Malaena → Fe def. An.

Treatment :

Deficult bec. Of multiple Bleeding sites.
Regular Fe therapy
Local Cuatary or Laser, Oestrogens

Hereditary Hemorrhagic Telangiectasia - vessel wall abnormalities

Ehlers – Donalos Dis :

AD. , Collagen defect
Fragile Bl. Ves. And organ memb. → Bleeding and organ rupture
Joint Hypermobility

Ehlers – Donalos Dis- vessel wall abnormalities


. Vit C def. → affect normal synthesis of collagen
. Perifollicular and patechial haemorrhage, sub‐ periosteal bleeding
. Diagnosis : Dietary History

scurvy- vessel wall abnormalities

Henoch – Schonlein Purpura (HSP)
. Children and young adults .
. Immune complex deposition in small ves. With vasculitis .
Often preceded by upper resp. inf.

Clinically :

1. Purpura: (raised) Buttocks and legs
2. Abdominal colicky pain and bleeding
3. Arthritis : Knees, Ankles .
4. Nephritis : 40% (May be later – 4 wks.)

Diagnosis : Clinical, Tissue biopsy ( IgA deposition in bl. Ves.)

Prognosis : good, Adverse if HTN, RF, Proteinuria > 1.5 g / day

Treatment :

Steroids for joint or GI involvment
Nephritis – pulse iv steroids ± immunosup.

Henoch – Schonlein Purpura (HSP)- vessel wall abnormalities

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