00:00 / 00:00
Autoimmune hemolytic anemia
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
Hemolytic disease of the newborn
Paroxysmal nocturnal hemoglobinuria
Pyruvate kinase deficiency
Sickle cell disease (NORD)
Folate (Vitamin B9) deficiency
Vitamin B12 deficiency
Anemia of chronic disease
Iron deficiency anemia
Vitamin K deficiency
Langerhans cell histiocytosis
Essential thrombocythemia (NORD)
Polycythemia vera (NORD)
Acute intermittent porphyria
Porphyria cutanea tarda
Disseminated intravascular coagulation
Von Willebrand disease
Monoclonal gammopathy of undetermined significance
Thrombotic thrombocytopenic purpura
Antithrombin III deficiency
Factor V Leiden
Protein C deficiency
Protein S deficiency
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
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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.
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