Lec 10Clinical Hematological

Assist Prof. Dr. Mudhir S. Shekha

Introduction

• Hematology: is the branch of medicine concerned with the study of the cause, prognosis, treatment, and prevention of diseases related to blood. It involves treating diseases that affect the production of blood and its components, such as blood cells, hemoglobin, blood proteins, bone marrow, platelets, blood vessels, spleen, and the mechanism of coagulation.

• Clinical Hematology: Is the study of diseases of the blood and bone marrow, and how to treat them.

• Hematopathology : is the study of diseases and disorders affecting blood cells, their production, and any organs and tissues involved in hematopoiesis, such as bone marrow, the spleen, and the thymus

•Blood disorders are diseases that affect one or more parts of the blood. The symptoms depend on the part of the blood affected

•blood-borne diseases →carried in the blood (HIV, hepatitis B, hepatitis C and viral hemorrhagic fevers)

•Many blood diseases →congenital and others may be acquired.

Blood disorders can be acute or chronic

• Acute blood disorders occur suddenly and last a shorttime. For example, rapid or excessive blood loss may cause acute anemia. Once the bleeding is stopped and blood levels return to normal, the anemia is cured.

• A chronic blood disorder is a disorder that develops slowlyor lasts a long time. Many chronic disorders, such as sickle cell anemia, cannot be cured. But the symptoms can be treated.

Example: Inflammations

Treatment

•Genetics→ ????•external causes→????

Treatment

• Genetics→ focused on managing the symptoms to keep the patient comfortable and help him lead a normal life (hemophilia) → in future (gene therapy).

• External causes→ treated by addressing the cause like blood malignancies by chaemotherapy and radiation to kill the malignant cells or marrow transplants and blood infusions

Blood Disease Symptoms• Skin will appear pale or bluish in color due to a lack

of blood flow, Petechiae, Blood blisters in the mouth, Swollen lymph nodes, Pallor, Pica

Decreased red blood cells and hemoglobin→• fatigue, weakness, and shortness of breath

Increased red blood cells→• headache and a red complexion (plethora)

Decreased white blood cells or immune system→

• cause recurrent fever and infections

Increased white blood cells or immune system→

• increased blood viscosity (thickening of the blood)

Decreased platelets or blood clotting factors→• abnormal bleeding and bruising

Increased platelets or blood clotting factors→

• thrombosis (inappropriate excessive blood clotting)

Red Blood Disorders

Anaemias• Anemia is defined as a reduction in the oxygen-carrying

capacity of the blood and usually is related to a decrease in number of circulating red blood cells (RBC) or to an abnormality in the hemoglobin (Hb) contained within the RBCs

• Because the main function of RBCs is oxygenation, anemia results in varying degrees of hypoxia

• Symptoms: feeling tired, weakness, shortness of breath or a poor ability to exercise, confusion, feeling like one is going to pass out, and an increased desire to drink fluids

• commonest →iron-deficiency anaemia, The MCV is central to the diagnosis of anemia.

• Iron deficiency causes microcytosis

• Vitamin B12 and folate deficiency →macrocytosis

• The anaemia of marrow suppression is often normocytic.

Causes of anaemia• Inherited

• Usually associated with reduced red cell survival (haemolytic anaemias):

• • Defects of haemoglobin, e.g. sickle cell, thalassaemia

• • Defects of red cell metabolism, e.g. pyruvate kinase deficiency

• • Defects of red cell membrane, e.g. hereditary spherocytosis

• • Red cell aplasia/aplastic anaemia

Causes of anaemiaAcquired

Reduced red cell production due to:

• Haematinic deficiency (iron, B12, folate, B6)

• Marrow replacement, e.g. by tumour (leukaemia, myeloma, lymphoma)

• Marrow aplasia

Causes of anaemiaIncreased red cell destruction (haemolytic anaemia) due to:

• Immune destruction

• Red cell fragmentation syndromes

• Chemical and physical agents

• Infections

• Paroxysmal nocturnal haemoglobinuria

Systemic illnesses:

• Anaemia of chronic disease

• Renal failure, liver, cardiac, endocrine disease

Classification of anemia by pathogenesis

Blood loss anemia

Hemolytic anemias

Disorders of hemoglobin

Hypo-proliferative anemias

Iron deficiency anemia

Glucose 6 phosphate dehydrogenaseDeficiency (G-6-PD deficiency)Drug Induced Immune mediated

Sickle cell anemiaThalassemia

Vitamin B12 DeficiencyPernicious anemiaFolic acid deficiencyAplastic anemia

(trauma and gastrointestinal bleeding among )

Anemia Classification• Size of RBC’s

• Microcytic (Small)

• Macrocytic (Large)

• Normocytic (Normal Size)

• Concentration of Hgb

• Hypochromic (Less)

• Hyperchromic (More)

• Normochromic (Normal)

• Microcytic / Hypochromic (MCV