HEMATOLOGY AND ONCOLOGY

CELLULAR ELEMENTS

THE RED CELLS

Erythropoiesis
• stem cell proerthroblast basophilic polychromat ophilic

orthocromatophil ic

reticulocy te

Mature RBCs

Monocyte Development

stem cell

monoblast

promonocyte

Monocyte

Lymphocyte Development

Stem cell

lymphoblast

prolymphocyte Lymphocyte

The production of platelets is THROMBOPOIESIS

Plasma

Plasma

Sites of formation of hematopoietic cells

Differentiation pathway of hematopoietic cells

) HEMOGLOBIN ) Hb

Different Hemoglobin Types
Hb type Embryonic type % at birth % After 2 years Major embryonic Hb are not present after 3rd month of gestation. 3% 95% 1.5-3.5% Embryonic Hb may be present at birth in small percentage. Fetal type(α2γ 2) 45-90% Adult type A1 (α2β 2) A2 (α δ ) 10-55% 0-1.5%

Proportions of the various human hemoglobin polypeptide chains .through early life

valueHematocrit

IRON DEFICIENCYANEMIA

Iron Metabolism

Spooning of the nails

Atrophic glossitis

HEMOLYTIC ANEMIA

BILIRUBIN METBOLISM

HEMOGLOBIN

GLOBIN

HEME

AMINOACIDS

IRON

PROTOPORPHYRIN

HEME OXYGENASE BILIVERDIN BILIVIRDIN REDUCTASE UNCONGUGATED BILIRUBIN

UNCONGUGATED BILIRUBIN

FREE UNCONGUGATED BILIRUBIN

ALBUMIN BOUND BILIRUBIN

ALBUMIN BOUND BILIRUBIN

DETACHED FROM ALBUMIN

LIGAND Y&Z PROTEIN SMOOTH ENDOPLASMIC R

URIDINE DIPHOSPHATEGLUCORYL TRANSFERASE E CONGUGATED BILIRUBIN

INTESTINE

URINE

CONGUGATED BILIRUBIN

B-GLUCURONIDASE E UNCONGUGATED BILIRUBIN

INTESTINAL BACTERIAL FLORA E-COLI STECOBILINOGEN

OXYDIZED ENTEROHEPATIC CIRCULATION STERCOBILIN

]G6PD deficiency[

]G6PD deficiency[

fava beans

SICKLE CELL ANEMIA

INHERITANCE

PATHOPHYSIOLOGY

)Bone infarction)head necrosis

THALASSEMIA SYNDROMES

Geographical distribution

MODE OF TRANSMISSION

PATHOPHYSIOLOGY OF ΒTHALASSEMIA MAJOR

CLINICAL MANIFESTATION OF Β - THALASSEMIA MAJOR

Target cells

RADIOLOGIC INVESTIGATION
Plain x ray skull • There is maxillary over growth, prominent widening of the diploic spaces, with hair standing on end appearance.

Desferal pump

HEMORRHAGIC DISEASES

Introduction

Blood within the vascular tree remains fluid throughout life

If a blood vessel is damaged a rapid localized

RESPONSE OF BLOOB CLOT FORMATION will occur.

Introduction

Failure of clotting leads to

MECHNISMIS OF HEMOSTASIS

Formation of a haemostatic plug
Primary homeostasis Aggregation adhesion fibrinogen GPIIB,IIIA

activation

release

Secondary homeostasis
FIBRIN CLOT FORMATION

Dr:Mohamed El-shanshory

Factor level activity &severity
type severe moderate mild Activity Type of hemorrhage % <1% 1 -5% Spontaneous;hemarthrosis; deep tissue hemorrhage Bleeding following mild to moderate trauma

5–25% Bleeding following moderate to severe trauma Gynacologic & obestatric hemorrhage

High risk 30-50% carrier female

Gene therapy

PURPURAS

Pathogenesis
• ITP is an autoimmune

Bleeding time

THROMBOCYTOPENIA

PURPURIC SPOTS

HENOCH – SCHOENLEIN PURPURA

BLOOD TRANSFUSION

GROWTH FACTORS

Apharesis