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Physiologic Overview
• The hematologic system consist of the blood and
the sites where blood is produced, including the bone
marrow and the reticulo endothelial
system(RES).Blood is a specialized organ that
differs from other organs in that it exists in a fluid
state. Blood is composed of plasma and various types
of cells. Plasma is the fluid portion of blood; it
contains various proteins, such as
albumin,globulin,fibrinogen,and other factors
necessary for clotting, as well as electrolytes, waste
products, and nutrients. About 55% 0f blood volume
is plasma.
BLOOD
The cellular component of blood consist of three primary
cell types:RBCs(red blood cells or
erythrocytes),WBCs(white blood cells or leukocytes),and
platelets(thrombocytes).These cellular components of blood
normally make up 40% to 45% of blood volume. Because
most blood cells have a short life span, the need for the
body to replenish its supply of cells is continuous; this
process is termed hematopoiesis.The primary site for
hematopoiesis is the bone marrow. During embryonic
development and in other conditions, the liver and spleen
may also be involved.
Under normal conditions, the adult bone marrow produces
about 175 billion RBCs,70 billion neutrophils(mature form of
a WBC),and 175 billion platelets each day. When the body
needs more blood cells, as in infection (when more RBCs
are required.Thus,under normal conditions, the marrow
responds to increased demand and released adequate
numbers of Cells into the circulation.
The volume of blood in humans is approximately 7% to
10% of the normal body weight and amounts to 5 to
6L.Circulating through the vascular system and serving as a
link between body or gans,the blood carries oxygen
absorbed from the lungs and nutrients absorbed from the
gastrointestinal tract to the body cells for cellular
metabolism to the lungs,skin,liver,and kidneys, where they
are transformed and eliminated from the body. Blood also
carries hormones, antibodies,and substances to their sites
of action or use.
To function, blood must remain in its normally fluid state.
Because blood is fluid, the danger always exists that trauma
can lead to loss of blood from the vascular system. To
prevents this, an intricate clotting mechanism is activated
when necessary to seal any leak in the blood vessels.
Excessive clotting is equally dangerous, because it can
obstruct blood flow to vital tissues. To prevent this, the body
has a fibrinolytic mechanism that eventually dissolves clots
(thrombi)formed within blood vessels. The balance between
these two systems, clot (thrombus) formation and clot
(thrombus) formation and clot (thrombus) dissolution or
fibrinolysis,is called homeostasis.
LEU KEM IA
Definition:
• Leukemia is a neoplastic
disorder of the blood-forming
tissue, and primarily targets the
spleen, lymph nodes, and bone
marrow.
• “Cancer of the blood” and
the most common form of
childhood Cancer.
Literally “white blood”, is a neoplastic proliferation of one
particular cell type (granulovytes, monocytes, lymphocytes,or
megakaryocytes). The defect originates in the hematopeoitic stem
cell, the myeloid, or the lymphoid stem cell. The lymphomas are
neoplasm of lymphoid tissue, usually derived from B lymphocytes.
Multiple myeloma is a malignancy of the most mature form of B
lymphocyte, the plasma cell.
The common feature of the leukemia’s is an unregulated
proliferation 0f WBCs in the bone marrow. In acute forms (or late
stages of chronic forms), the proliferation of leukemic cells leaves
little room for normal cell production. There can also be a
proliferation of cells in the liver and spleen (extramedullary
hematopoiesis). With acute forms, there can be infiltration of other
organs, such as the meninges, lymph nodes, gums, and skin. The
cause of leukemia is not fully known, but there is some evidence
that genetic influence and viral pathogenesis may be involved.
Bone marrow damage from radiation exposure or from chemicals
such as benzene and alkylating agents (eg, melphalan {Alkeran})
can cause leukemia.
Pa thophysio logy:
• All types of leukemias result from the
abnormal development of leukocytes in the
bone marrow. Maturational arrest occurs, and
a proliferative, clonal population of cells
result. A variety of defects promote the clonal
expansion of leukemic cells. These defects
include an abnormal proliferative potential,
defects in terminal differentiation, and
defective apoptosis. The increased
proliferative potential is caused by the
activation of oncogenes or the inactivation of
tumor suppressor genes. Leukemia cutis is
thought to result from a local proliferation of
the leukemic cells within the skin.
The pathophysiology underlying the specific migration of leukemic
cells to the skin is not clear. In the case of human T-cell leukemia
virus type I (HTLV-I)–induced leukemia, it may be due to the
abundant expression of the CC chemokine receptor 4 (CCR4) on
the cell surface of the leukemic cells. The ligands thymus and
activation-regulated chemokine (TARC/CCL17) and macrophage-
derived chemokine (MDC/CCL22) are present in the skin and may
explain the predilection of adult T-cell leukemia to involve the skin.
Evidence also suggests that the presence of T-cell–related
antigens on the cell surface of leukemic cells in acute monocytic
leukemia (AML-M5) in patients with leukemia cutis may promote
selective homing to the skin. Additionally, one small study of 18
cases of myelomonocytic leukemia cutis patients showed
cutaneous lymphocyte-associated antigen (CLA) staining in 14
(78%) of 18 cases. The presence of CLA may confer a specific
tropism to the skin in these leukemic cells.
ASSOCIATED MEDICAL
CONDITIONS
1. Acute Myeloid Leukemia (AML)
• AML, results from a defect in the
hematopoietic stem cell that differentiates
into all myeloid cells: monocytes,
granulocytes (neutrophils, basophils,
eosinophils), erythrocytes, and platelets.
All age group are affected; the incidence
rises with age, with a peak incidence at
age 60 years. AML is the most common
nonlymphocytic leukemia.
Acute Myeloid Leukemia
2.Chronic Myeloid
leukemia (CML)