Вы находитесь на странице: 1из 20

Morphologic Anemias?

What are the major features of


Anemias Due to Defective
Hemoglobin Synthesis
(Microcytic Anemias)
Iron Deficiency Anemia?

What are the Major features of B


Thalassemia minor?

Asymptomatic
Morphology and Lab features

Microcytic hypo chromic or normochromic red cells, target cells


Erythrocytosis
Low MCV and MCHC, or low MCV and normal MCHC
Hb electrophoresis HbA1 normal, increase in HbA2, +/- increase in
HbF

Characterized by reduced synthesis (alpha+ thalassemias) or


absent synthesis (Alpha thalassemia) of alpha-globin
chains.
Since there are normally four alpha globin genes, the
severity of the clinical syndromes shows a great variation,
depending upon the number of affected alpha globin genes.

Give me an overview of B
thalassemia minor?

Two genes from each parent


Deletion of genes most common
Reduced synthesis of alpha chains.
Excess (Hb Bart) or (Hb H) chains precipitate
causing red cell damage.

What are the clinical syndromes


for A Thalassemia?

Clinical Genotype

Disease

Silent carrier-a Iaa

None

Trait-a/-a

Similar to B Thal
minor

HbH Disease

Severe,
resembles B
Thal intermedia

Hydrops

Lethal in utero
Hb baits

How do you diagnose A


thalassemia?

Hb electrophoresis is normal in silent carriers


and trait
HbH and Barts (fast), inclusions in HbH

Exclude other causes of microcytic hypochromic


anemia

Treatment: Similar to B Thalassemia

What is the Pathophysiology of


Megaloblastic anemia?

Defective DNA synthesis and normal RNA and


protein synthesis
Myeloid cells and non-hematopoietic rapidly
proliferating cells are affected
Ineffective hematopoiesis (RPI < 2)

What is the etiology and clinical


features of Megaloblastic
anemia?

Etiology:
Vitarnin B12 and folate deficiencies, drug induced (chemotherapy).

Clinical Features:

Symptoms and signs of anemia in general.


Yellow skin discoloration
Dyspepsia and glossitis
neurological symptoms (Vitamin B12)

Peripheral blood Pancytopenia


Macroovalocytes (MCV >100), Howell-Jolly bodies and hyper
segmented neutrophils

Bone marrow morphology Megaloblastic changes, giant bands

What is the structure and source


as well as the requirements for
Vitamin B12 Deficiency?

Structure and source- Cobalamine. Exclusive source


is dietary animal products (e.g. eggs, milk, meat).

Requirements: 2-3 ug/day,


70% of uptake is absorbed (terminal ileum)
stores are 5000 ug mostly in liver (50%)
kidney and heart (50%) enough for several years.

What are the laboratory features


of Vitamin B12 deficiency?

Low serum Vitamin B12 level


Low red cell folate level
Increased urinary excretion of methylmalonic acid
Ineffective erythropoiesis: increased indirect
bilirubin and urobilinogen, increased LDH enzyme,
increased serum iron levels.

What is pernicious anemia?

What are the major features, the


structure and source and the
requirements for folate
deficiency?

Вам также может понравиться