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B-thal major - severe form of b-thal, rbc abnormal

pallor - unusual loss of brightness

homozygous - 2 identical allele of a gene

abdominal distention - swollen beyond normal size, usually related to being bloated

splenomegaly - enlarge of spleen

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ten-year-old boy

scheduled blood transfusion

diagnosed to have beta thalassaemia

major (homozygous beta thalassaemia). -

His condition was associated with pallor,

loss of appetite, unusual tiredness, abdominal distension

and shortness of breath.

He developed splenomegaly,

which got progressively worse and increased his

transfusion requirements.

occasionally

hospitalised due to infections.

parents were

alive and well, second of four children.

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B- thal

secondary infection

hemolytic anemia

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--> Jaundice = produce excees amount of biiliruin , cause yellowish apperance on skin and conjunctiva

--> tachycardia = increased heart rate over 100 bpm

--> hepatosplenomegaly = enlargment of liver and spleen

--> hypochromia = less coulouration due to less hb

--> Target cell = presence of stain at the central pallor of RBC,appears as a dark ring sorrounding a dark entral spot, certain kind of aneamia. Codocytes

Microcytosis = RBC size smaller than normal range

--> reticulocytosis = immature in RBC that still contain remnants of RNA release from the bone marrow into the blood stream

# cytosis = increase , penia = decrease

--> Polychromasia = Variation in color of RBC on aperipheral blood smear

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--> severe pallor,

mild jaundice

hepatosplenomegaly.

tachycardia.

severe anaemia,

hypochromia, microcytosis,polychromasia

target cells,

circulating nucleated red blood cells and reticulocytosis

Haematocrit (Hct)

Hb- A, Hb-A2 and Hb-F \

MCV, MCH, MCHC, RDW,

Platlet count and WBC count

Serum ferritin, folate and B12

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--> severe pallor = high insoluble globin in RBC, RBC become less flexible and prone to destruction, Leads low level of Hb

@ can also due to emotional stress

--> mild jaundice = Elevated level of billirubin due to excessive destruction of RBC.

--> hepatosplenomegaly. = over workload of the liver and the spleen by handling the destruction of RBC

--> tachycardia. = lack of O2, Heart pumps harder to achive sufficient blood flow

--> severe anaemia,= due to lack of beta-globin protein > Lack of RBC in body

--> hypochromia, microcytosis,polychromasia

target cells, = Common symptoms that indicate b-thal. Excess alpha globin chain can damage RBC membrane, leads to chnage in the flexibilty and deformability of the cell membrane.

--> circulating nucleated red blood cells and = RBC in cell still contain nucleus

--> reticulocytosis = release more immatature erythroblast to compensate low RBC count.

--> Haematocrit (Hct) = % by Volume of RBC in blood . Decrease functional RBC, increase reticulocyte in RBC, Beta chain decrease, Hb decrease

--> Hb- A, = consist of 2 alpha and 2 beta chain , in patient has decrease beta chain

Hb-A2 and Hb-F = excess alpha chain, tend to bind with gamma and delta chain. Alpha-gamma = Hb-F, Alpha-delta = Hb-A2

# Measured by electrophoresis

MCV + decrease indicate microcytic RBC

MCH, =ndecrease indicate hypochromic RBC

MCHC,= normal but no OK!!! bcs polychromacia

RDW, = Normal but not OKKK, bcs all blood cell are microcytic

Platlet count and WBC count = RBC low due to destruction of RBC

Serum ferritin, = protein that stores iron, normal indcates no iron deficiency

folate and B12 = important for RBC devolepment

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ten-year-old boy

kids, poor hygene, prone to get infection

low immmunity

scheduled blood transfusion

having transfusion dependent thal, severe ineffective eryhtropoiesis(development of rbc) , leads to hemolysis rbc, for maintain hb

diagnosed to have beta thalassaemia

major (homozygous beta thalassaemia).

-both parent as carrier,

b- thal -> alpha globin excess

His condition was associated with pallor,loss of appetite, unusual tiredness, abdominal distension

and shortness of breath.

-pallor -> less hb, (anemia)

loss of appetite - lack of nutrition , infection, due to anemia,malignancy

abdominal distention - splenomegaly

SOB- due to hb-f keep holding o2, high affinity o2 - need more o2

He developed splenomegaly,

which got progressively worse and increased his

transfusion requirements

-splenic enlarge,increase destruction of rbc(anemia), to maintain adequate o2 to deliver to tissue

occasionally

hospitalised due to infections.

-overload iron in body - reduce immune response- innate immunity

-toxic

parents were

alive and well, second of four children

inherited disease, parents as a thal carrier

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B-Thal

hypochromic microcytic Anemia

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Anti-Duffy Ab - antibodies against the Duffy blood group Ags implicated in hemolytic transfusion reaction and hemolytic disease of the fetus and newborn

Iron CHelation Therapy - therapy used to balance a rate of iron accumulation from blood transfusion

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Pretransfusion test

Anti-Duffy Ab

blood transfusion safely done

maintained on folic acid supplement

iron Chelation therapy

risk of future child having same problem

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Pretransfusion test - to scan for antibodies against blood group antigens

Anti-Duffy Ab - unexpected, delayed reaction

blood transfusion safely done

maintained on folic acid supplement - one of treatment for thalassemia (other treatments include bone marrow transplant and blood transfusion, splenotomy)

iron Chelation therapy - reduce iron load in transfused blood

risk of future child having same problem - 50% carrier, 25% patient, 25% normal

prevention: know blood status before getting married/having kids