Coagulation disorders: Pathology review
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Coagulation disorders: Pathology review
Hematological system
Anemias
Iron deficiency anemia
Beta-thalassemia
Alpha-thalassemia
Sideroblastic anemia
Anemia of chronic disease
Lead poisoning
Hemolytic disease of the newborn
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
Autoimmune hemolytic anemia
Pyruvate kinase deficiency
Paroxysmal nocturnal hemoglobinuria
Sickle cell disease (NORD)
Hereditary spherocytosis
Anemia of chronic disease
Aplastic anemia
Fanconi anemia
Megaloblastic anemia
Folate (Vitamin B9) deficiency
Vitamin B12 deficiency
Fanconi anemia
Diamond-Blackfan anemia
Heme synthesis disorders
Acute intermittent porphyria
Porphyria cutanea tarda
Lead poisoning
Coagulation disorders
Hemophilia
Vitamin K deficiency
Platelet disorders
Bernard-Soulier syndrome
Glanzmann's thrombasthenia
Hemolytic-uremic syndrome
Immune thrombocytopenic purpura
Thrombotic thrombocytopenic purpura
Mixed platelet and coagulation disorders
Von Willebrand disease
Disseminated intravascular coagulation
Heparin-induced thrombocytopenia
Thrombosis syndromes (hypercoagulability)
Antithrombin III deficiency
Factor V Leiden
Protein C deficiency
Protein S deficiency
Antiphospholipid syndrome
Lymphomas
Hodgkin lymphoma
Non-Hodgkin lymphoma
Leukemias
Chronic leukemia
Acute leukemia
Leukemoid reaction
Leukemoid reaction
Dysplastic and proliferative disorders
Myelodysplastic syndromes
Polycythemia vera (NORD)
Myelofibrosis (NORD)
Essential thrombocythemia (NORD)
Langerhans cell histiocytosis
Mastocytosis (NORD)
Plasma cell dyscrasias
Multiple myeloma
Monoclonal gammopathy of undetermined significance
Waldenstrom macroglobulinemia
Hematological system pathology review
Microcytic anemia: Pathology review
Non-hemolytic normocytic anemia: Pathology review
Intrinsic hemolytic normocytic anemia: Pathology review
Extrinsic hemolytic normocytic anemia: Pathology review
Macrocytic anemia: Pathology review
Heme synthesis disorders: Pathology review
Coagulation disorders: Pathology review
Platelet disorders: Pathology review
Mixed platelet and coagulation disorders: Pathology review
Thrombosis syndromes (hypercoagulability): Pathology review
Lymphomas: Pathology review
Leukemias: Pathology review
Plasma cell disorders: Pathology review
Myeloproliferative disorders: Pathology review
Assessments
Coagulation disorders: Pathology review
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Transcript
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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