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Hematological system
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
Langerhans cell histiocytosis
Mastocytosis (NORD)
Essential thrombocythemia (NORD)
Myelodysplastic syndromes
Myelofibrosis (NORD)
Polycythemia vera (NORD)
Leukemoid reaction
Disseminated intravascular coagulation
Heparin-induced thrombocytopenia
Von Willebrand disease
Antiphospholipid syndrome
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
Myeloproliferative disorders: Pathology review
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Laboratory value | Result |
Hemoglobin | 19.0 g/dL |
Hematocrit | 57% |
Erythrocytes | 7.2 million/mm3 |
Leukocytes | 12,900/mm3 |
Platelets | 440,000/mm3 |
A 70 year old female named Jenny is brought by her husband to the emergency department complaining of blurred vision and headache. Her face appears plethoric and her husband says that Jenny has been complaining of extreme itchiness after showers for the last few days. She has no significant past medical history. CBC shows increased hematocrit and slightly increased platelets. Uric acid is also increased. Next to Jenny, there’s a 65 year old male named Seth that came in with fatigue and progressive weight loss due to early satiety. Past medical history is unremarkable. Clinical examination reveals a very enlarged spleen. CBC shows pancytopenia and peripheral blood smear shows teardrop cells.
Both Jenny and Seth suffer from myeloproliferative neoplasms. These are a group of malignant neoplasms characterized by proliferation of the bone marrow cells from the myeloid lineage. That includes RBCs, platelets, as well as granulocytes, which include neutrophils, basophils, mast cells, and eosinophils. Each disorder can potentially cause proliferation of all of the myeloid cells, but they’re classified based on the dominant cell line involved. So there’s polycythemia vera, for RBCs, essential thrombocythemia for platelets, chronic myeloid leukemia, or CML, for granulocytes, and the odd one out, primary myelofibrosis, which doesn’t really have a dominant cell line, but instead is characterized by bone marrow fibrosis.
Okay, now CML is associated with the 9:22 translocation, which is when there’s fusion of the BCR gene on chromosome 22 and the ABL tyrosine kinase gene on chromosome 9. This is called the Philadelphia chromosome. Now, in this video, let’s focus on the myeloproliferative disorders that are not associated with Philadelphia chromosome, or the “Philadelphia chromosome negative myeloproliferative disorders,” like polycythemia vera, essential thrombocythemia, and primary myelofibrosis. All right, so let’s take a closer look at these myeloproliferative disorders, starting with polycythemia vera, where there’s an increase in RBC production. It typically begins with a mutation in a single hematopoietic stem cell, which gives rise to RBCs, WBCs, and platelets.
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