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Assignment On Thalassemia

Thalassemia is an inherited autosomal recessive blood disorder characterized by impaired synthesis of the alpha or beta globin chains that make up hemoglobin. It was initially identified in people near the Mediterranean Sea. There are two main types: alpha thalassemia affects alpha chain synthesis while beta thalassemia affects beta chain synthesis. The severity depends on how many defective genes are inherited. In beta thalassemia, beta chain production is depressed resulting in anemia and destruction of red blood cells.

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392 views

Assignment On Thalassemia

Thalassemia is an inherited autosomal recessive blood disorder characterized by impaired synthesis of the alpha or beta globin chains that make up hemoglobin. It was initially identified in people near the Mediterranean Sea. There are two main types: alpha thalassemia affects alpha chain synthesis while beta thalassemia affects beta chain synthesis. The severity depends on how many defective genes are inherited. In beta thalassemia, beta chain production is depressed resulting in anemia and destruction of red blood cells.

Uploaded by

Munira
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© © All Rights Reserved
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Download as DOCX, PDF, TXT or read online on Scribd
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Thalassemia

The alpha and beta Thalassemia are inherited autosomal recessive disorder that can
cause an impaired rate of synthesis of one of the two chains, alpha or beta of Hb A.
The disorder was named thalassemia, which is derived from the Greek word for sea,
because it was defined initially in people with origins near the Mediterranean Sea.
Alpha thalassemia-alpha chain is affected.
Beta thalassemia-synthesis of beta globin chains are slowed and defective
Alpha and beta thalassemia is characterized as major or minor, depending on how
many chains that control the synthesis of alpha or beta chains is defected and
whether the defects are inherited homozygously or heterozygously. Normally two
genes control beta chain synthesis and four genes control alpha chain synthesis.
The number of defected genes determines the severity of the disorder.
Beta thalassemia
The fundamental defect in beta thalassemia is the uncoupling of alpha and beta
chain synthesis. Beta chain production is depressed- moderately in the
heterozygous form, beta thalassemia minor and severely in homozygous form,
beta thalassemia major (also called Cooleys anemia). Beta thalassemia
intermedia may also happen when both of the beta globin genes are mutated, but
the mutations are less severe than those that typically cause beta thalassemia
major. People with this condition usually have moderately severe anemia and
sometimes require regular blood transfusions. Depression of beta chain synthesis
results in erythrocytes having a reduced amount of hemoglobin and accumulation of
alpha free chains. The free alpha chains are unstable and easily precipitate in the
cell. Most erythroblast that contains precipitates are destroyed by mononuclear
phagocytosis, resulting in ineffective erythropoiesis and anemia. Some of the
precipitated cells do enter blood stream, but they are prematurely destroyed in the
spleen resulting in mild hemolytic anemia. (Kathryn L. McCance, 2014)

Lymphomas
Hodgkin Lymphoma

Is a cancer of the blood and bone marrow

Is one of the most curable forms of cancer

Is named for Dr. Thomas Hodgkin, who first noted a trend of cancer cases in
the lymph nodes in 1832. The disease was called Hodgkin's disease until it
was officially renamed Hodgkin lymphoma in the late 20th century.

Treatment includes radiation, chemotherapy or both, depending on individual


patient factors.

How Does Hodgkin Lymphoma Develop?


Hodgkin lymphoma starts when an abnormal change to a white cell (called
a lymphocyte) causes it to become a lymphoma cell.

Lymphoma cells grow and form masses, usually in the lymph nodes, located
throughout our bodies in the lymphatic system.

Lymphoma cells can also gather in other areas of the body where lymphoid
tissue is found.

Hodgkin lymphoma is distinguished from other types of lymphoma by the


presence of Reed-Sternberg cells (named for the scientists who first identified
them). Other cells associated with the disease are called Hodgkin cells.

If untreated, the cancerous cells crowd out normal white cells, and
the immune system can't guard against infection effectively.

Non-Hodgkin lymphoma (NHL)

Is a type of cancer that affects the lymphatic system

Generally develops in the lymph nodes and lymphatic tissues. In some cases,
NHL involves bone marrow and blood.

Isn't just one diseaseit's actually a diverse group of blood cancers that share
a single characteristic in how they develop

NHL has many different subtypes which are either indolent (slow growing) or
aggressive (fast growing).

How Does NHL Develop?

NHL usually starts with an abnormal change in a white cell in a lymph


node or lymphoid tissue called a lymphocyte. It can start in one of three
major types of lymphocytes:

B lymphocytes (B cells), which produce antibodies to help combat infections

T lymphocytes (T cells), which have several functions, including helping B


lymphocytes make antibodies

Natural killer (NK) cells, which attack virus-infected cells or tumor cells

About 85 percent of NHL cases start in the B cells. Your doctor plans your treatment
according to the type of cell your NHL developed in.
The abnormal lymphocyte grows out of control and produces more
abnormal cells like it.

These abnormal lymphocytes (lymphoma cells) accumulate and form masses


(tumors). If NHL isn't treated, the cancerous cells crowd out normal white
cells, and the immune system can't guard against infection effectively.

NHL that develops in or spreads to other areas of the body where lymphoid
tissue is found, such as the spleen, digestive tract and bone marrow, is
called primary extranodal lymphoma.

NHL is classified into more than 30 different subtypes. Doctors classify


the NHL subtypes into categories that describe how rapidly or slowly the
disease is progressing:
o

Aggressive NHL

Indolent (slow-growing) NHL (Lymphoma)

COMMON BLOOD DISORDERS


Anemia
Common Types of Anemia
Iron-deficiency anemia
Vitamin-deficiency anemia
Aplastic anemia
Hemolytic anemia
Sickle cell anemia

Bleeding Disorders
Hemophilia
Blood Cancers
Leukemia
Lymphoma
Myeloma
Blood Clots
Deep Vein Thrombosis
Thrombotic Thrombocytopenic Purpura (American Society of Haematology)

Bibliography
American Society of Haematology. (n.d.). Retrieved from
http://www.hematology.org/Patients/Blood-Disorders.aspx
Kathryn L. McCance, . E. (2014). Pathophysiology- The Biologic Basis for disease in
Children and Adults. Elsevier Mosby. Retrieved from
https://books.google.com.pk/books?
id=3QgyAgAAQBAJ&pg=PA1069&dq=thalassemia+pathophysiology&hl=en&
sa=X&ved=0ahUKEwjzhN3opfQAhVsLcAKHVhICJgQ6AEINzAD#v=onepage&q=thalassemia
%20pathophysiology&f=false
Lymphoma. (n.d.). Retrieved from Leukemia and Lymphoma Society:
https://www.lls.org/lymphoma

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