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Questions and Answers
What is a universal blood donor blood type?
What is a universal blood donor blood type?
Rh- individuals will produce antibodies after the first exposure to Rh+ RBCs.
Rh- individuals will produce antibodies after the first exposure to Rh+ RBCs.
False
What is the responsible agent in Haemolytic Disease of the Newborn (HDN)?
What is the responsible agent in Haemolytic Disease of the Newborn (HDN)?
IgG antibodies
The common complication from a transfusion gone wrong is _____ transfusion reaction.
The common complication from a transfusion gone wrong is _____ transfusion reaction.
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Which of the following is a macrocytic anaemia?
Which of the following is a macrocytic anaemia?
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What does APTT stand for in coagulation studies?
What does APTT stand for in coagulation studies?
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Which of the following is a potential risk factor for developing a deep vein thrombosis (DVT)?
Which of the following is a potential risk factor for developing a deep vein thrombosis (DVT)?
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_____ is the condition when there is a deficiency of red blood cells or hemoglobin in the blood.
_____ is the condition when there is a deficiency of red blood cells or hemoglobin in the blood.
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What is the primary factor involved in Coomb’s Test?
What is the primary factor involved in Coomb’s Test?
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Lead poisoning can cause microcytic anaemia.
Lead poisoning can cause microcytic anaemia.
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Match the following blood cell conditions with their characteristics:
Match the following blood cell conditions with their characteristics:
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What is a pulmonary embolism?
What is a pulmonary embolism?
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Which drug is a glycoprotein IIb/IIIa receptor antagonist?
Which drug is a glycoprotein IIb/IIIa receptor antagonist?
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What does tranexamic acid treat?
What does tranexamic acid treat?
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Acute leukemias are less aggressive than chronic leukemias.
Acute leukemias are less aggressive than chronic leukemias.
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Which type of leukemia typically affects immature WBCs?
Which type of leukemia typically affects immature WBCs?
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Hodgkin’s Lymphoma contains ______ cells.
Hodgkin’s Lymphoma contains ______ cells.
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What is a common associated mutation with Polycythemia Rubra Vera?
What is a common associated mutation with Polycythemia Rubra Vera?
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Which of the following conditions is characterized by fibrosis of the bone marrow?
Which of the following conditions is characterized by fibrosis of the bone marrow?
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What clinical feature is associated with Multiple Myeloma?
What clinical feature is associated with Multiple Myeloma?
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What is the major issue with Myelodysplastic Syndrome?
What is the major issue with Myelodysplastic Syndrome?
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What is pulmonary embolism?
What is pulmonary embolism?
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Which antiplatelet medication irreversibly inhibits COX?
Which antiplatelet medication irreversibly inhibits COX?
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What condition is associated with excess clotting and can lead to organ damage?
What condition is associated with excess clotting and can lead to organ damage?
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Leukemias arise from the ______ and affect ______?
Leukemias arise from the ______ and affect ______?
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What are the two types of lymphomas?
What are the two types of lymphomas?
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Acute leukemias are generally less aggressive than chronic leukemias.
Acute leukemias are generally less aggressive than chronic leukemias.
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Match the following types of hematological malignancies with their descriptions:
Match the following types of hematological malignancies with their descriptions:
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What is the characteristic feature of Multiple Myeloma?
What is the characteristic feature of Multiple Myeloma?
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Essential Thrombocytosis is characterized by overproduction of ______.
Essential Thrombocytosis is characterized by overproduction of ______.
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What mutation is 95% associated with Polycythemia Rubra Vera?
What mutation is 95% associated with Polycythemia Rubra Vera?
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Which blood type is considered the universal blood donor?
Which blood type is considered the universal blood donor?
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Rh- individuals will produce antibodies if exposed to Rh+ red blood cells.
Rh- individuals will produce antibodies if exposed to Rh+ red blood cells.
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What is the responsible agent in Haemolytic Disease of the Newborn (HDN)?
What is the responsible agent in Haemolytic Disease of the Newborn (HDN)?
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What is the treatment for Rh- pregnant women to prevent HDN?
What is the treatment for Rh- pregnant women to prevent HDN?
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What does agglutination indicate during blood typing?
What does agglutination indicate during blood typing?
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Name one common complication that can arise from blood transfusions.
Name one common complication that can arise from blood transfusions.
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Microcytic anaemia is characterized by decreased ______ production.
Microcytic anaemia is characterized by decreased ______ production.
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Match the types of anaemia with their respective causes:
Match the types of anaemia with their respective causes:
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What is the main mechanism of haemostasis?
What is the main mechanism of haemostasis?
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Vitamin K is not required for clotting factor synthesis.
Vitamin K is not required for clotting factor synthesis.
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What is Virchow’s triad?
What is Virchow’s triad?
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What type of blood cell is primarily involved in the immune response during an allergic reaction?
What type of blood cell is primarily involved in the immune response during an allergic reaction?
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Study Notes
Blood Typing
- Blood groups are named according to the antigen on the red blood cells.
- If a red blood cell has the antigen, the plasma won't have the antibody.
- O- is the universal blood donor and AB+ is the universal plasma donor.
- RhD (Rhesus D) is a blood group protein attached to red cells.
- Everyone is either Rh+ or Rh-.
- Rh- individuals will not produce antibodies unless previously exposed to Rh+ red blood cells.
Haemolytic Disease of the Newborn (HDN)
- HDN usually occurs in the second pregnancy due to previous exposure.
- IgG antibodies are responsible for causing HDN (past exam question).
- RhD immunoglobulin is administered to Rh- pregnant women to mop up any foetal red blood cells and prevent maternal antibody production.
Coomb’s Test
- Detects antibodies on the surface of red blood cells
- Direct Antiglobulin Test/Coomb's Test
- Identifies antibodies on the surface of red blood cells.
- Used in cases of autoimmune haemolysis, drug-induced haemolysis, and alloimmune haemolysis.
- Indirect Antiglobulin Test/Coomb's Test
- Detects antibodies in the serum.
- Utilized for pretransfusion testing and prenatal antibody screening (HDN).
- Direct Antiglobulin Test/Coomb's Test
Determining Blood Groups
- Cell Typing
- Determines the ABO antigen present on the red blood cells.
- The patient's red blood cells are mixed with known antibodies.
- Agglutination indicates a positive result.
- Serum Testing
- Determines the ABO antibodies present in the patient serum.
- The patient's serum is mixed with known red blood cells.
- Agglutination indicates a positive result.
- Consider transfusions when Hb < 70.
Transfusions Gone Wrong
- Common complications include:
- Acute haemolytic transfusion reaction (within 24 hours).
- Delayed haemolytic transfusion reaction (days to months).
- Graft vs Host Disease (donor cells/organ attacks the host, often seen in immunosuppressed hosts).
Anaemia
- A deficiency of red blood cells or haemoglobin in the blood.
- Symptoms include fatigue, shortness of breath, pallor, palpitations, sore tongue, arrhythmia.
- Anaemia is classified into four types.
- Microcytic
- Normocytic
- Macrocytic
- Some bad jokes.
Microcytic Anaemia
- Decreased haemoglobin production.
- Types:
- Thalassaemia
- Faulty haemoglobin synthesis.
- Common where malaria is endemic.
- Various types and severity depending on the genetic mutation.
- More uniform in cell size and shape, much lower haemoglobin compared to MCV.
- Alpha: common in Asia, Africa.
- Beta: more severe, Mediterranean.
- Anaemia of Chronic Disease.
- Iron Deficiency
- Presentation: cold feet/hands, hair loss, brittle nails, unusual cravings.
- Lead Poisoning.
- Sideroblastic
- Unable to use iron effectively to make haemoglobin.
- Thalassaemia
Normocytic Anaemia
- Impaired blood cell production.
- Increased reticulocytes:
- Haemolysis
- Bloods may have high reticulocytes, low haptoglobin, high lactate dehydrogenase.
- Causes include metabolic abnormalities (e.g. G6DP deficiency), haemoglobin abnormalities (e.g. sickle cell, thalassaemia), membrane abnormalities (e.g. hereditary spherocytosis), immune-mediated, mechanical, drugs.
- Acute blood loss.
- Haemolysis
- Normal/decreased reticulocytes.
- Bone marrow disease.
- Anaemia of chronic disease.
- Kidney failure.
Macrocytic Anaemia
- Impaired blood cell production.
- Megaloblastic
- B12 deficiency
- Less common, slow onset, neural degeneration.
- Folate deficiency
- More common nutritional deficiency, weeks onset, may be drug-related.
- Presentation: numbness/tingling, balance/gait, confusion, diarrhoea nausea.
- B12 deficiency
- Pernicious Anaemia
- Intrinsic factor (IF) produced in the stomach is needed to absorb Vitamin B12.
- A lack of IF most commonly occurs due to an autoimmune attack against intrinsic factor and parietal cells.
Coagulation
- The process of blood clotting.
- Haemostasis
- Injury.
- Local smooth muscle contraction.
- Platelet adhesion/activation/aggregation.
- Secondary - Coagulation: fibrinogen to fibrin.
- Coagulation Pathway
- Negative feedback, degrade Va and VIIIa.
- Tiny quantities of thrombin amplify activation of other clotting factors and co-factors.
- Related Pharmacology
- Hereditary thrombophilia conditions
- Defect/deficiency of anticoagulant proteins (e.g. antithrombin III, protein C, protein S).
- Genetic mutation increased tendency towards thrombosis (e.g. factor V Leiden (APC resistance), elevated clotting factors).
- Hereditary haemophilia conditions
- Haemophilia A: Deficiency of VIII.
- Haemophilia B: Deficiency of IX.
- Hereditary thrombophilia conditions
- Unhelpful Movie References
- This part is a bit irrelevant but it helps to picture the process of coagulation in the context of a movie.
- Haemostasis
Anticoagulants
- Enoxaparin (LMWH)
- Longer t1/2.
- Subcutaneous administration.
- Potentiates antithrombin action on X.
- Factor Xa inhibitor (common factors of 10 in descending order: 10, 5, 2, 1).
- Apixaban and Rivaroxaban
- Factor Xa inhibitors.
- Heparin
- IV/subcutaneous.
- Emergency reversal - protamine sulphate.
- Antithrombin III binds to heparin.
- Count down from 12 and skip 10 (since it's common): 12, 11, 9, 8.
- Ok in pregnancy.
- Warfarin
- Oral, onset delayed.
- Vitamin K antagonist.
- Maintain with INR (variable, ~2-3.5).
- Teratogenic.
- Dabigatran
- Direct oral anticoagulant (DOAC), no INR, similar efficacy to warfarin.
- Bind reversibly to thrombin.
Coagulation Studies
- APTT: intrinsic + common pathway.
- TT: common pathway.
- PT: extrinsic + common pathway.
- INR: International Normalized Ratio, derived from PT, standardizes testing.
- Mixing Test: to determine the cause of a prolonged PT/APTT, the patient’s plasma is mixed with control plasma.
- Correction with mixing indicates a factor deficiency.
- Non-correction with mixing suggests the presence of an inhibitor.
Virchow’s Triad
- A patient who smokes, has hypertension, a BMI of 30, and a history of breast cancer at 45 years old is at risk for developing a deep vein thrombosis (DVT).
- The patient also recently underwent a hip replacement.
- These factors are all related to Virchow’s triad:
- Hypercoagulability (increased tendency for blood clotting - e.g. smoking, hypertension, history of breast cancer).
- Endothelial damage (damage to the lining of blood vessels - e.g. hip replacement).
- Stasis (slow blood flow - e.g. obesity).
- All of these factors increase the risk of developing DVT.
Deep Vein Thrombosis (DVT)
- A clot forms in a vein.
- Commonly in the lower extremities.
Pulmonary Embolism
- Pulmonary embolism (PE) is a blockage of the pulmonary artery, typically caused by a blood clot (deep vein thrombosis, DVT) from the legs or pelvis.
Antiplatelets
- Abciximab is a glycoprotein IIb/IIIa receptor antagonist.
- Clopidogrel inhibits ADP-induced platelet activation by blocking ADP binding to the P2Y12 receptor.
- Aspirin irreversibly inhibits cyclooxygenase (COX), decreasing thromboxane production and platelet aggregation.
-
Von Willebrand factor (vWF) is synthesized by endothelial cells and stored in cytoplasmic granules.
- It binds to collagen and activates when bound, allowing it to bind to surface receptors.
- vWF also serves as a carrier for Factor VIII.
- Deficiency in vWF may lead to a mildly prolonged activated partial thromboplastin time (APTT), with normal international normalized ratio (INR) and platelet count.
Fibrinolysis and Fibrinolytics/Anti-fibrinolytics
- Tranexamic acid is used to treat heavy bleeding and is generally short-acting.
- Alteplase is recombinant human tissue plasminogen activator (tPa) utilized in acute ST-elevation myocardial infarction (STEMI) and life-threatening PE.
- Disseminated Intravascular Coagulation (DIC) is characterized by excessive clotting, leading to ischemia and organ damage, and excess bleeding, resulting in shock and hypotension.
Hematological Malignancies
- Hematological malignancies include leukemias, lymphomas, myeloid diseases.
Leukemias and Lymphomas
- Leukemias arise from the bone marrow and affect immature white blood cells (WBCs).
- Lymphomas originate from lymph nodes and affect mature lymphocytes.
- Leukemias can metastasize to lymph nodes, but are still considered leukemias due to their origin.
Leukemias
- Leukemias are characterized by unregulated proliferation of immature and non-functional leukocytes in the bone marrow.
- The buildup of these immature leukocytes crowds out normal cells, leading to decreased amounts of functional cells. This is more common in acute leukemias.
- Acute leukemias are more aggressive but often curable.
- Chronic leukemias are often asymptomatic and rarely curable.
-
Clinical Manifestations of Leukemias:
- Anemia: Syncope, angina, dyspnea, fatigue.
- Thrombocytopenia: Increased bleeding and bruising.
- Leukopenia: Increased infections.
- Systemic: Weight loss, fever, night sweats, loss of appetite.
- Organs: Hepatosplenomegaly, bone pain, lymphadenopathy.
Types of Leukemias
- Acute Myeloid Leukemia (AML)
- Chronic Myeloid Leukemia (CML)
- Acute Lymphoid Leukemia (ALL)
-
Chronic Lymphoid Leukemia (CLL)
- Auer rods are associated with AML.
- Smudge cells are associated with CLL.
Lymphomas
- Lymphomas involve the accumulation of non-functional lymphocytes in the lymph nodes. These cells do not produce antibodies.
-
Two types of Lymphomas:
- Hodgkin's lymphoma: Contains Reed-Sternberg cells (owl eyes).
- Non-Hodgkin's lymphoma: Does not contain Reed-Sternberg cells.
- Hodgkin's lymphoma typically has a better prognosis.
-
Clinical Manifestations of Lymphomas:
- B symptoms: Fever, night sweats, and weight loss.
- Lymphadenopathy.
Myeloid Diseases
- 1. Polycythemia Rubra Vera
- 2. Essential Thrombocytosis
- 3. Primary Myelofibrosis
- 4. Multiple Myeloma
- 5. Myelodysplastic Syndrome
Polycythemia Rubra Vera
- Polycythemia Rubra Vera is characterized by an overproduction of red blood cells.
- 95% association with a JAK2 mutation.
-
Signs and Symptoms:
- Thrombotic events.
- Bleeding (intracranial and gastrointestinal).
- Pruritis after a hot shower.
- Erythromelalgia.
-
Management:
- Phlebotomy: Until hematocrit (Hct) is less than or equal to 0.45.
- Prophylactic aspirin: To prevent thrombotic events.
- Cytoreductive therapy: Using hydroxyurea or ruxolitinib.
-
Diagnosis:
- Increased RBCs, resulting in high hemoglobin, hematocrit, or red blood cell mass.
- Bone marrow biopsy with trilineage growth.
- JAK2 mutation.
- Decreased erythropoietin (EPO) levels (minor criterion).
Essential Thrombocytosis
- Essential thrombocytosis is characterized by an overproduction of platelets.
- 50% of patients have a JAK2 mutation (can also be caused by CALR or MPL mutations).
-
Clinical Features:
- Thrombotic events.
- Spontaneous abortion.
- Acute gouty arthritis.
- Headaches.
- Treatment: Low-dose aspirin and hydroxyurea.
Primary Myelofibrosis
- Primary myelofibrosis is a myeloproliferative neoplasm that leads to fibrosis of the bone marrow.
- 50% association with JAK2 mutation.
- It presents with hepatosplenomegaly due to extramedullary hematopoiesis.
- Hyperproliferative stage: Increased WBC count and platelet count.
- Pancytopenic stage: Decreased production of all blood cell lineages.
- Blood film: Teardrop cells (cells have to squeeze out of the fibrotic bone marrow).
- Bone marrow biopsy: Dry aspiration (punctio sicca).
- Treatment: Stem cell transplantation (younger patients) or supportive treatment.
Multiple Myeloma
- Multiple myeloma is characterized by uncontrolled proliferation of plasma cells that produce monoclonal immunoglobulins.
-
Signs and Symptoms (CRAB):
- Hypercalcemia
- Renal Failure
- Anemia
- Bony lesions
- Blood film: Rouleaux bodies.
- Bone marrow: Clusters of plasma cells.
- Urine test: Bence Jones proteins (produced by neoplastic cells).
Myelodysplastic Syndrome
- Myelodysplastic syndrome is characterized by malfunctioning stem cells that lead to ineffective maturation of all cell lineages, resulting in cytopenia of multiple cell lines.
- Usually idiopathic, affecting elderly patients.
-
Blood film:
- Refractory anemia: Doesn’t respond to usual therapy such as iron, folate, or vitamin B12.
- Hyposegmented nuclei
- Large agranular platelets
- Bone marrow: Hypercellular and dysplastic.
- Treatment: Possible allogeneic stem cell transplantation, but generally supportive (eg., blood transfusions, granulocyte colony-stimulating factor).
Blood Typing
- Blood types are determined by the presence of antigens on red blood cells.
- If a red blood cell has the antigen, the plasma does not have the antibody.
- O- is the universal blood donor.
- AB+ is the universal plasma donor.
RhD
- RhD is a blood group protein attached to red blood cells.
- Everyone is either Rh+ or Rh-.
- Rh- individuals will not produce antibodies unless previously exposed to Rh+ red blood cells.
Haemolytic Disease of the Newborn (HDN)
- HDN is usually seen in the second pregnancy due to prior exposure to Rh+ red blood cells.
- IgG antibodies are responsible for HDN.
- RhD immunoglobulin administered to Rh- pregnant women helps prevent maternal antibody production.
Coomb’s Test
- Detects antibodies on the surface of red blood cells or in serum.
- Direct Coomb’s test determines if antibodies are present on RBCs.
- Indirect Coomb’s test determines the presence of antibodies in the serum.
Determining Blood Groups
- Cell typing determines the ABO antigens present on red blood cells using known antibodies. Agglutination indicates a positive result.
- Serum testing determines the ABO antibodies present in patient serum using known red blood cells. Agglutination indicates a positive result.
Transfusions
- Transfusions are typically considered when hemoglobin levels are below 70 g/L.
- Common complications include acute haemolytic transfusion reaction, delayed haemolytic transfusion reaction, and graft vs. host disease.
Anaemia
- Anaemia is a deficiency of red blood cells or haemoglobin in the blood.
- It can present with various symptoms like fatigue, shortness of breath, pallor, palpitations, sore tongue, arrhythmia.
Microcytic Anaemia
- Characterized by decreased haemoglobin production.
- Thalassaemia: Faulty haemoglobin synthesis.
- Anaemia of chronic disease: Typically found in chronic inflammatory conditions.
- Iron deficiency: Occurs due to insufficient iron levels.
- Lead poisoning: Leads to impairment of haemoglobin production.
- Sideroblastic anaemia: Inability to use iron effectively for haemoglobin synthesis.
Normocytic Anaemia
- Characterized by normal or decreased red blood cell size.
- Increased reticulocytes: Indicates increased red blood cell production.
- Haemolysis: Destruction of red blood cells.
- Acute blood loss: Results in a sudden decrease in red blood cells.
- Bone marrow disease: Affects the production of red blood cells.
- Anaemia of chronic disease: Commonly seen in chronic inflammatory conditions.
- Kidney failure: Can affect red blood cell production.
Macrocytic Anaemia
- Characterized by increased red blood cell size.
- Megaloblastic anaemia: Caused by impaired blood cell production due to B12 or folate deficiency.
- Macronormoblastic anaemia: Larger red blood cells with generally normal appearances.
Coagulation
-
Haemostasis is the process of stopping bleeding. It involves a series of steps:
- Injury.
- Local smooth muscle contraction.
- Platelet adhesion, activation, and aggregation.
- Coagulation: fibrinogen to fibrin.
- Negative feedback mechanisms degrade Va and VIIIa to prevent excessive clotting.
Anticoagulants
- Heparin: Anticoagulant that enhances the action of antithrombin, typically administered intravenously or subcutaneously. Protamine sulfate is used for emergency reversal.
- Warfarin: Oral anticoagulant that acts as a vitamin K antagonist, requiring regular monitoring with international normalized ratio (INR).
- Dabigatran: Direct oral anticoagulant (DOAC) that binds reversibly to thrombin, not requiring INR monitoring and offering similar efficacy to warfarin.
- Enoxaparin: Low-molecular-weight heparin (LMWH) with a longer half-life, given subcutaneously, potentiates antithrombin’s effect on factor X.
Coagulation Studies
- APTT (activated partial thromboplastin time): Measures the intrinsic and common coagulation pathways.
- TT (thrombin time): Measures the common coagulation pathway.
- PT (prothrombin time): Measures the extrinsic and common coagulation pathways.
- INR (international normalized ratio): A standardized measure derived from PT to improve consistency across different laboratories.
- Mixing test: Used to determine the cause of prolonged PT or APTT. If mixing with control plasma corrects the result, it suggests a factor deficiency. If it does not correct, it indicates an inhibitor.
Virchow’s Triad
- Virchow’s triad is a helpful framework understanding risk factors for deep vein thrombosis.
- The three main components are:
- Hypercoagulability: A tendency towards clotting.
- Endothelial damage: Damage to the lining of blood vessels.
- Abnormal blood flow: Stasis or slow blood flow.
Deep Vein Thrombosis (DVT)
- DVT is the formation of a blood clot in a vein, typically in the lower extremities.
- Risk factors include smoking, hypertension, obesity, and previous history of cancer.
Pulmonary Embolism
- Occurs when a blood clot breaks off from a vein, usually in the legs or pelvis, travels through the bloodstream, and blocks a pulmonary artery in the lung.
Antiplatelets
- Clopidogrel inhibits ADP induced platelet activation by blocking the P2Y12 receptor.
- Aspirin irreversibly inhibits COX, decreasing thromboxane production and reducing platelet aggregation.
- Abciximab is a glycoprotein IIb/IIIa receptor antagonist, preventing platelets from binding to fibrinogen and aggregating.
- Von Willebrand Factor (vWF) is synthesized by endothelial cells and released into the bloodstream, where it binds to collagen and plays a role in platelet adhesion and aggregation.
Fibrinolysis and Fibrinolytics/Anti-Fibrinolytics
- Alteplase is a recombinant human tPA (tissue plasminogen activator) used to treat acute STEMI and life-threatening PE by dissolving clots.
- Tranexamic Acid is used to treat heavy bleeding, short-term, by inhibiting the breakdown of fibrin.
- Disseminated Intravascular Coagulation (DIC) is a serious condition where excessive clotting consumes platelets and clotting factors, leading to both thrombosis and bleeding.
Hematological Malignancies
- Leukemias arise from the bone marrow and affect immature white blood cells (WBCs).
- Lymphomas arise from lymph nodes and affect mature lymphocytes.
- Multiple Myeloma involves an overproduction of B cells.
- Essential Thrombocytosis involves an overproduction of platelets.
- Polycythemia Rubra Vera involves an overproduction of red blood cells (RBCs).
- Primary Myelofibrosis results in fibrosis of the bone marrow.
- Myelodysplastic Syndrome involves malfunctioning stem cells that lead to ineffective maturation of blood cells.
Leukemias vs. Lymphomas
- Leukemias affect immature white blood cells in the bone marrow.
- Lymphomas affect mature lymphocytes in lymph nodes.
- Leukemias can metastasize to lymph nodes, but they are still classified as leukemias due to their origin in the bone marrow.
Leukemias
- Leukemias are characterized by unregulated proliferation of immature or non-functional leukocytes in the bone marrow.
- Acute leukemias are more aggressive but often curable.
- Chronic leukemias are often asymptomatic but rarely curable.
- Clinical manifestations of leukemias include anemia, thrombocytopenia, leukopenia, systemic symptoms (weight loss, fever, night sweats, appetite loss), and organomegaly.
Leukemias - 4 Types
- Acute Myeloid Leukemia (AML): Characterized by Auer rods.
- Chronic Myeloid Leukemia (CML): Often associated with the Philadelphia chromosome.
- Acute Lymphoid Leukemia (ALL): Most common leukemia in children.
- Chronic Lymphoid Leukemia (CLL): Characterized by smudge cells.
Lymphomas
- Lymphomas are characterized by non-functional lymphocytes accumulating in lymph nodes.
- Hodgkin's Lymphoma contains Reed-Sternberg cells (owl eyes) and usually has a better prognosis.
- Non-Hodgkin's Lymphoma does not contain Reed-Sternberg cells.
- Clinical manifestations of lymphomas include B symptoms (fever, night sweats, weight loss) and lymphadenopathy.
Myeloid Diseases
- These are characterized by disorders affecting the myeloid lineage of blood cells.
- Polycythemia Rubra Vera (PRV): High red blood cell count (Hb, Hct, or RBC mass), often associated with a JAK2 mutation.
- Essential Thrombocytosis (ET): High platelet count, often associated with JAK2, CALR, or MPL mutations.
- Primary Myelofibrosis (PMF): Fibrosis of the bone marrow, often associated with a JAK2 mutation.
- Multiple Myeloma (MM): Uncontrolled proliferation of plasma cells producing monoclonal immunoglobulins.
- Myelodysplastic Syndrome (MDS): Malfunctioning stem cells leading to ineffective blood cell maturation.
Polycythemia Rubra Vera (PRV)
- Clinical features: Thrombotic events, bleeding, pruritis after hot shower, erythromelalgia.
- Management: Phlebotomy, prophylactic aspirin, cytoreductive therapy (hydroxyurea, ruxolitinib).
- Diagnosis: Increased RBC count, bone marrow biopsy with trilineage growth, JAK2 mutation, decreased EPO levels.
Essential Thrombocytosis (ET)
- Clinical features: Thrombotic events, spontaneous abortion, acute gouty arthritis, headaches.
- Treatment: Low dose aspirin and hydroxyurea.
Primary Myelofibrosis (PMF)
- Clinical features: Hepatosplenomegaly, hyperproliferative stage followed by pancytopenic stage.
- Blood film: Teardrop cells.
- Bone marrow biopsy: Dry aspiration (punctio sicca).
- Treatment: Stem cell transplantation (younger patients) or supportive treatment.
Multiple Myeloma (MM)
- Clinical features: CRAB symptoms (hypercalcemia, renal failure, anemia, bone lesions).
- Blood film: Rouleaux bodies.
- Bone marrow: Clusters of plasma cells.
- Urine test: Bence Jones proteins.
Myelodysplastic Syndrome (MDS)
- Clinical features: Refractory anemia (doesn't respond to usual therapy), hyposegmented nuclei, large agranular platelets.
- Bone marrow: Hypercellular and dysplastic.
- Treatment: Allogeneic stem cell transplantation (possible) or supportive treatment (blood transfusions, granulocyte colony stimulating factor).
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Test your knowledge on blood types, disorders, and transfusion reactions in this comprehensive quiz. Questions cover universal blood donors, haemolytic disease of the newborn, and complications from blood transfusions. Ideal for students studying hematology or related medical fields.
