HEMATOPOIETIC

AND LYMPHOID
SYSTEMS
Dr. Aroosha
Introduction
◦ The hematopoietic and lymphoid systems are affected by a wide spectrum of diseases. One way to
organize these disorders is based on whether they primarily affect red cells, white cells, or the hemostatic
system, which includes platelets and clotting factors
◦ Although these divisions are useful, in reality the production, function, and destruction of red cells, white
cells, and components of the hemostatic system are closely linked, and pathogenic derangements
primarily affecting one cell type or component of the system often lead to alterations in others
◦ For example, in certain conditions B cells make autoantibodies against components of the red cell
membrane. The opsonized red cells are recognized and destroyed by phagocytes in the spleen, which
becomes enlarged. The increased red cell destruction causes anemia, which in turn drives a compensatory
hyperplasia of red cell progenitors in the bone marrow
 RED CELL
DISORDERS
Anemia
◦ Disorders of red cells can result in anemia or, less commonly, polycythemia (an increase in red cells also
known as erythrocytosis). Anemia is defined as a reduction in the oxygen-transporting capacity of
blood, which usually stems from a decrease in the red cell mass to subnormal levels.
◦ Anemia can result from bleeding, increased red cell destruction, or decreased red cell production.
◦ In some cases entities overlap occurs, for example, in thalassemia where reduced red cell production and
early destruction give rise to anemia.
◦ Classification of anemia according to morphology reveals three types
◦ Microcytic (iron deficiency, thalassemia)
◦ Macrocytic (folate or vitamin B12 deficiency)
◦ Normocytic but with abnormal shapes (sickle cell disease)
Continued…
◦ ANEMIA OF BLOOD LOSS: HEMORRHAGE
◦ With acute blood loss exceeding 20% of blood volume, the immediate threat is hypovolemic shock rather than
anemia. If the patient survives, hemodilution begins at once and achieves its full effect within 2 to 3 days; only then
is the full extent of the red cell loss revealed. The anemia is normocytic and normochromic. Recovery from blood
loss anemia is enhanced by a compensatory rise in the erythropoietin level, which stimulates increased red cell
production within 5-7 days
◦ HEMOLYTIC ANEMIAS
◦ Normal red cells have a life span of about 120 days. Anemias caused by accelerated red cell destruction are termed
hemolytic anemias. Features shared by all uncomplicated hemolytic anemias include (1) a decreased red cell life
span, (2) a compensatory increase in erythropoiesis, and (3) the retention of the products of degraded red cells
(including iron) by the body.
◦ Destruction of red cells can occur within the vascular compartment (intravascular hemolysis) or within tissue
macrophages (extravascular hemolysis).
HEMOLYTIC
ANEMIAS
SICKLE CELL ANEMIA
◦ The hemoglobinopathies are a group of hereditary disorders caused by inherited mutations that lead to
structural abnormalities in hemoglobin
◦ Sickle cell anemia, the prototypical (and most prevalent) hemoglobinopathy, stems from a mutation
in the β-globin gene that creates sickle hemoglobin (HbS).
◦ Normal hemoglobins are tetramers composed of two pairs of similar chains
◦ HbS is produced by the substitution of valine for glutamic acid at the sixth amino acid residue of β-
globin
◦ Sickle cell anemia is the most common familial hemolytic anemia in the world.
◦ Two major consequences arise from the sickling of red cells First, the red cell membrane damage and
dehydration caused by repeated episodes of sickling produce a chronic hemolytic anemia. ). Second, red
cell sickling produces widespread microvascular obstructions, which result in ischemic tissue damage.
THALASSEMIA
◦ The thalassemias are inherited disorders caused by mutations that decrease the synthesis of α- or β-globin
chains.
◦ As a result, there is a deficiency of Hb.
◦ The mutations that cause thalassemia are particularly common among populations in Mediterranean,
African, and Asian regions in which malaria is endemic.
◦ TYPES
◦ When we talk about different “types” of thalassemia, we might be talking about one of two things: the specific part
of hemoglobin that is affected (usually either “alpha” or “beta”), or the severity of thalassemia, which is noted by
words like trait, carrier, intermedia, or major.
◦ Hemoglobin, which carries oxygen to all cells in the body, is made of two different parts, called alpha and beta.
When thalassemia is called “alpha” or “beta,” this refers to the part of hemoglobin that isn’t being made. If either
the alpha or beta part is not made, there aren’t enough building blocks to make normal amounts of hemoglobin.
Low alpha is called alpha thalassemia. Low beta is called beta thalassemia.
Continued…
SYMPTOMS
◦ Fatigue
◦ Weakness
◦ Pale or yellowish skin
◦ Facial bone deformities
◦ Slow growth
◦ Abdominal swelling
◦ Dark urine
Paroxysmal Nocturnal Hemoglobinuria
◦ Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder worthy of mention because it is the only
hemolytic anemia that results from an acquired somatic mutation in myeloid stem cells.
◦  The median age of diagnosis is 35-40 years of age, with occasional cases diagnosed in childhood or
adolescence.
◦ PNH occurs when mutations of a gene called PIG-A occur in a bone marrow stem cell. Stem cells give rise to
all the mature blood elements including red blood cells , which carry oxygen to our tissues; white blood cells ,
which fight infection; and platelets, which are involved in forming blood clots. In PNH, the affected stem cell
passes the PIG-A mutation to all cells derived from the abnormal stem cell. Cells harboring PIG-A mutations are
deficient in a class of proteins called GPI-anchored proteins.
◦ Certain GPI-anchored proteins protect red blood cells from destruction, some are involved in blood clotting, and
others are involved in fighting infection. The majority of PNH-related issues, including destruction of red blood
cells (hemolytic anemia), blood clots (thrombosis), and infection, result from a deficiency of these proteins.
Immunohemolytic Anemias
◦ A condition in which the body’s immune system stops red blood cells from forming or causes them to
clump together.
◦ Normally when your immune system spots foreign invaders like bacteria and viruses, it makes proteins
called antibodies to attack them. When you have AIHA, your immune system makes antibodies that
mistakenly attack your own red blood cells.
◦ Types
◦ Autoimmune hemolytic anemia is classified as
◦ Primary AIHA: Appears by itself
◦ Secondary AIHA: Affects you because you have another illness. 
Malaria
◦ Malaria is caused by a single-celled parasite of the genus plasmodium. The parasite is transmitted to
humans most commonly through mosquito bites.
◦ Plasmodium parasites are spread by the bite of infected female Anopheles mosquitoes, which feed on
human blood in order to nourish their own eggs.
◦ an infected mosquito injects immature forms of the parasite, called sporozoites, into the person’s
bloodstream. The sporozoites are carried by the blood to the liver, where they mature into forms known
as schizonts
◦ Over the next one to two weeks each schizont multiplies into thousands of other forms known as
merozoites. The merozoites break out of the liver and reenter the bloodstream, where they invade red
blood cells, grow and divide further, and destroy the blood cells in the process.
THANK YOU