Coagulation disorders: Pathology review
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Coagulation disorders: Pathology review
Hematological system
Anemias
Autoimmune hemolytic anemia
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
Hemolytic disease of the newborn
Hereditary spherocytosis
Paroxysmal nocturnal hemoglobinuria
Pyruvate kinase deficiency
Sickle cell disease (NORD)
Fanconi anemia
Folate (Vitamin B9) deficiency
Megaloblastic anemia
Vitamin B12 deficiency
Alpha-thalassemia
Anemia of chronic disease
Beta-thalassemia
Iron deficiency anemia
Lead poisoning
Sideroblastic anemia
Anemia of chronic disease
Aplastic anemia
Diamond-Blackfan anemia
Fanconi anemia
Dysplastic and proliferative disorders
Langerhans cell histiocytosis
Mastocytosis (NORD)
Essential thrombocythemia (NORD)
Myelodysplastic syndromes
Myelofibrosis (NORD)
Polycythemia vera (NORD)
Leukemoid reaction
Leukemoid reaction
Mixed platelet and coagulation disorders
Disseminated intravascular coagulation
Heparin-induced thrombocytopenia
Von Willebrand disease
Thrombosis syndromes (hypercoagulability)
Antiphospholipid syndrome
Antithrombin III deficiency
Factor V Leiden
Protein C deficiency
Protein S deficiency
Hematological system pathology review
Coagulation disorders: Pathology review
Extrinsic hemolytic normocytic anemia: Pathology review
Heme synthesis disorders: Pathology review
Intrinsic hemolytic normocytic anemia: Pathology review
Leukemias: Pathology review
Lymphomas: Pathology review
Macrocytic anemia: Pathology review
Microcytic anemia: Pathology review
Mixed platelet and coagulation disorders: Pathology review
Myeloproliferative disorders: Pathology review
Non-hemolytic normocytic anemia: Pathology review
Plasma cell disorders: Pathology review
Platelet disorders: Pathology review
Thrombosis syndromes (hypercoagulability): Pathology review
Assessments
Coagulation disorders: Pathology review
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Transcript
Content Reviewers
At the hematology ward, there’s Braden, a 5 year old male, who developed prolonged bleeding after circumcision.
His mother is worried because he has a history of recurrent hemarthrosis after minor falls.
Family history reveals a relative of his mother who suffered from bleeding diathesis.
Now, there’s also a 3 day old preterm baby, called Harlow, who is bleeding severely from the umbilicus.
Her mother states that she did not get the standard care after delivery. CBC, PT and PTT are ordered for both patients.
They both have normal platelet count.
Now, Braden has normal PT but elevated PTT, while Harlow has both PT and PTT elevated.
Both Braden and Harlow are suffering from a hemostasis disorder.
Hemostasis disorders, also known as bleeding disorders, can be broadly divided into three groups.
The first includes problems with primary hemostasis, which is the formation of the platelet plug, so they are also called platelet disorders.
The second group includes problems with secondary hemostasis, which is making a strong fibrin clot through activation of the intrinsic, extrinsic and common coagulation pathways, and so they’re known as coagulation disorders.
And the last group includes disorders that affect both primary and secondary hemostasis and are known as mixed platelet and coagulation disorders.
Let’s focus on coagulation disorders that are usually due to a decrease in the number of clotting factors and causes include hemophilia and vitamin K deficiency.
So, let’s look at hemophilia first.
They are a group of inherited bleeding disorders caused by deficiencies in various coagulation factors.
Hemophilia A and B are X-linked recessive disorders so a high yield fact is that they almost exclusively affect males while females are carriers.
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Kumar & Clark's Clinical Medicine" Saunders (2009)
- "Establishing the Prevalence and Prevalence at Birth of Hemophilia in Males" Annals of Internal Medicine (2019)
- "The Diagnosis and Management of Congenital Hemophilia" Seminars in Thrombosis and Hemostasis (2012)
- "Haematology" Scion Pub Limited (2010)
- "Vitamin K-dependent carboxylase. Haematologia (Budap)" Vermeer C, De boer-van den berg MA. (1985)
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