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.

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

secondary infection

hemolytic anemia

--> 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

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

@ 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

hypochromic microcytic Anemia