Patterns of skin bleeding
Petechial purpura Palpable purpura Ecchymosis
Minor bleeding into dermis, that is Flat & non- Raised & non-blanching, able to be touched or Extensive bleeding into deeper layers of the skin
blanching felt upon palpation Joints → hemarthroses
Petechiae are typically found with Indicates vasculitis secondary to serious disease
thrombocytopenia or platelet dysfunctions
Vessels wall abnormalities
• Dominantly inherited condition
• Clinical pictures
o Telangiectasia & aneurysm in fingertips, face, tongue, nasal passages, lung & GIT
o Pulmonary arterio-venous malformations → causes arterial hypoxemia, due to right-to-left shunt → predispose to
Hereditary paradoxical embolism → resulting in stroke or cerebral abscess
hemorrhagic o Recurrent bleeding as epistaxis
telangiectasia o Iron deficiency due to occult GI bleeding
(HHT) • Management
o Difficult due to multiple bleeding points
o Regular iron therapy → allow the marrow to compensate for blood loss
o Local cautery or laser therapy → may prevent single lesions from bleeding
• Autosomal dominant caused by → defect in type 3 collagen → fragile blood vessels & organ membranes → bleeding & organ
ruptures
• Clinical pictures
Ehlers-Danlos o Joint hypermobility
disease o Skin changes & facial appearance are typical
• Diagnoses should be considered when there is history of bleeding with normal laboratory tests
• Vitamin C deficiency affects normal synthesis of collagen & results in bleeding disorder
• Characterized by
Scurvy o Perifollicular & petechial hemorrhage
o Bruising & subperiosteal bleeding
• Key to diagnosis is the Dietary history
, Abnormal platelet
• Either acquired or congenital
• The most common cause of acquired is iatrogenic, e.g. → NSAIDs (aspirin), clopidogrel, ticagrelor that prevent arterial
thrombosis
Platelet dysfunctions • Congenital functional defects (generally rare) as
o Storage pool disease
o Glanzmann disease → severe bleeding requires mechanical measures + antifibrinolytics + platelet transfusion
• All congenital cause mild bleeding after trauma or surgery rare spontaneously except Glanzmann disease
Thrombocytopenia
• ↓ Production
o Marrow hypoplasia
▪ Childhood bone marrow failure syndromes → Fanconi’s anemia, dyskeratosis congenita & amegakaryocytic thrmo
▪ Idiopathic aplastic anemia & Drug induced → cytotoxic & antimetabolites
o Marrow infiltration → Leukemia, myeloma, osteopetrosis, carcinoma, myelofibrosis & lysosomal storage disease (Gaucher’s)
o Hematinic deficiency → Vit B12 &/or folate deficiency
o Familial (Macro-) thrombocytopenia
▪ Myosin heavy chain abnormalities → Alport’s syndrome, Fechtner’s syndrome & May-Hegglin anomaly
▪ Bernard-Soulier syndrome & Montreal platelet syndrome
▪ Wiskott-Aldrich syndrome (small platelets)
Causes
• ↑ Consumption (imp.)
o Immune mechanisms
▪ Idiopathic thrombocytopenic purpura (ITP)
▪ Neonatal alloimmune thrombocytopenia & post-transfusion purpura
▪ Drug-associated → Quinine, Vancomycin & Heparin
o Coagulation activation → Disseminated intravascular coagulation (DIC)
o Mechanical pooling → Hypersplenism
o Thrombotic microangiopathies
▪ Hemolytic uremic syndrome (HUS), Liver disease & Pre-eclampsia
▪ Thrombotic thrombocytopenic purpura (TTP)
Clinical • Platelet count fall below 20k → Spontaneous bleeding
picture • Purpura & spontaneous bruising are characteristic, but there may be also oral, nasal, GI or genitourinary bleeding
• Severe thrombocytopenia < 10k → Retinal hemorrhage & potentially fatal IC bleeding (rare)
Investigations • Blood film (most imp.) & BM aspiration
Management • Treat underlying causes & platelet transfusion (rarely indicated)
Petechial purpura Palpable purpura Ecchymosis
Minor bleeding into dermis, that is Flat & non- Raised & non-blanching, able to be touched or Extensive bleeding into deeper layers of the skin
blanching felt upon palpation Joints → hemarthroses
Petechiae are typically found with Indicates vasculitis secondary to serious disease
thrombocytopenia or platelet dysfunctions
Vessels wall abnormalities
• Dominantly inherited condition
• Clinical pictures
o Telangiectasia & aneurysm in fingertips, face, tongue, nasal passages, lung & GIT
o Pulmonary arterio-venous malformations → causes arterial hypoxemia, due to right-to-left shunt → predispose to
Hereditary paradoxical embolism → resulting in stroke or cerebral abscess
hemorrhagic o Recurrent bleeding as epistaxis
telangiectasia o Iron deficiency due to occult GI bleeding
(HHT) • Management
o Difficult due to multiple bleeding points
o Regular iron therapy → allow the marrow to compensate for blood loss
o Local cautery or laser therapy → may prevent single lesions from bleeding
• Autosomal dominant caused by → defect in type 3 collagen → fragile blood vessels & organ membranes → bleeding & organ
ruptures
• Clinical pictures
Ehlers-Danlos o Joint hypermobility
disease o Skin changes & facial appearance are typical
• Diagnoses should be considered when there is history of bleeding with normal laboratory tests
• Vitamin C deficiency affects normal synthesis of collagen & results in bleeding disorder
• Characterized by
Scurvy o Perifollicular & petechial hemorrhage
o Bruising & subperiosteal bleeding
• Key to diagnosis is the Dietary history
, Abnormal platelet
• Either acquired or congenital
• The most common cause of acquired is iatrogenic, e.g. → NSAIDs (aspirin), clopidogrel, ticagrelor that prevent arterial
thrombosis
Platelet dysfunctions • Congenital functional defects (generally rare) as
o Storage pool disease
o Glanzmann disease → severe bleeding requires mechanical measures + antifibrinolytics + platelet transfusion
• All congenital cause mild bleeding after trauma or surgery rare spontaneously except Glanzmann disease
Thrombocytopenia
• ↓ Production
o Marrow hypoplasia
▪ Childhood bone marrow failure syndromes → Fanconi’s anemia, dyskeratosis congenita & amegakaryocytic thrmo
▪ Idiopathic aplastic anemia & Drug induced → cytotoxic & antimetabolites
o Marrow infiltration → Leukemia, myeloma, osteopetrosis, carcinoma, myelofibrosis & lysosomal storage disease (Gaucher’s)
o Hematinic deficiency → Vit B12 &/or folate deficiency
o Familial (Macro-) thrombocytopenia
▪ Myosin heavy chain abnormalities → Alport’s syndrome, Fechtner’s syndrome & May-Hegglin anomaly
▪ Bernard-Soulier syndrome & Montreal platelet syndrome
▪ Wiskott-Aldrich syndrome (small platelets)
Causes
• ↑ Consumption (imp.)
o Immune mechanisms
▪ Idiopathic thrombocytopenic purpura (ITP)
▪ Neonatal alloimmune thrombocytopenia & post-transfusion purpura
▪ Drug-associated → Quinine, Vancomycin & Heparin
o Coagulation activation → Disseminated intravascular coagulation (DIC)
o Mechanical pooling → Hypersplenism
o Thrombotic microangiopathies
▪ Hemolytic uremic syndrome (HUS), Liver disease & Pre-eclampsia
▪ Thrombotic thrombocytopenic purpura (TTP)
Clinical • Platelet count fall below 20k → Spontaneous bleeding
picture • Purpura & spontaneous bruising are characteristic, but there may be also oral, nasal, GI or genitourinary bleeding
• Severe thrombocytopenia < 10k → Retinal hemorrhage & potentially fatal IC bleeding (rare)
Investigations • Blood film (most imp.) & BM aspiration
Management • Treat underlying causes & platelet transfusion (rarely indicated)
