Presentation

GROUP 2( ROLL NO. 28-55)

Scenario:

 A 19-year-old female student presented with the complaint of

enlargement of abdomen for last few years. She lives in a dormitory.
 There is no history of pain , nausea or fever. She has normal menstrual cycle. There is no family
history of any chronic disease .
 Upon examination, her BP is 110/70 mmHg, Temperature 36.5 C, Respiratory Rate 16 /min. She
looks icteric and pale . There is costal retraction while breathing .
 Upon palpation of her abdomen, the Liver is 2 finger and Spleen is 3 finger enlarged. Bowel
sounds are normal.
 Investigations reveal an Hb of 9.8 g, WBC 9.9 x 10x3 per micro litre, Platelet 28000 per micro
litre, MCH = 10 pg, MCV = 59 fl, and MCHC = 28.9.
Learning Objectives

1. Difficult terms
2. Symptoms & Reasons
3. Differential Diagnosis
4. Normal Values of Hb, MCH, MCV, MCHC
5. Anemia
6. Hemolytic Anemia
 Causes, Pathology, Histopathology, Investigation & Treatment
Difficult Terms

 Dormitory: A large sleeping room with several beds accommodating large number of people.
 Icteric: Yellowish / pale looking
 Costal retraction: Inward movement of abdomen in costal area while breathing; due to reduced air pressure
inside chest.
 MCH: stands for Mean Corpuscular Hemoglobin and is average amount of hemoglobin contained in a
person's RBCs.
 MCHC: Stands for Mean Corpuscular Hb Concentration and is the amount of Hb an RBC has, relative to
cell's volume.
 MCV: Stands for Mean Corpuscular Volume which is average size and volume of an RBC.
Symptoms and Their Reasons

37, 46 & 47
Differential Diagnosis

38, 48
Normal values of Hb, MCH, MCV, MCHC

RBC index Normal Value

HB Males – 14 to 18 g/dl, Female – 12 to 16 g/dl

MCV 80 to 100 femtoliter

MCH 27 to 31 picograms/cell

MCHC 32 to 36 grams/deciliter (g/dL) or 320 to 360 grams per liter (g/L)

Anemia

28, 29
Classification
of Anemia
Hemolytic Anemia
1. Causes
Hemolytic Anemia
2. Pathology

33, 49
Hemolytic Anemia
3. Histopathology
Histopathological features of hemolytic anemia may include:
 Spleen Changes: Histologically, the spleen in hemolytic anemia may show signs of congestion,
increased activity of macrophages (phagocytic cells), and the presence of hemosiderin, which is a
pigment formed from the breakdown of hemoglobin.
 Bone Marrow Changes: Hemolytic anemia can lead to an increased production of RBCs in the
bone marrow (erythroid hyperplasia) . This may result in an increase in erythroid precursors in the
bone marrow.
 Peripheral Blood Smear: A peripheral blood smear may reveal characteristic changes, such as the
presence of spherocytes (small, round RBCs), polychromasia (immature RBCs), and
reticulocytotic (increased numbers of reticulocytes, which are young RBCs).
Hemolytic Anemia
3. Histopathology (Cont.)

 Bilirubin Deposits: The breakdown of hemoglobin in hemolytic anemia can lead to an excess
of bilirubin, which may deposit in tissues, causing jaundice. Histologically, you might see
bilirubin deposits in various tissues.
 Hemosiderosis: Chronic hemolysis can lead to hemosiderosis, the accumulation of iron in
tissues. This can be seen histologically as brown pigment (hemosiderin) in various organs.
 Vascular Changes: In some cases, the excessive destruction of RBCs can lead to vascular
changes, such as increased fragility of blood vessels, which may contribute to features like
splenomegaly
Hemolytic Anemia
4. Investigations
Laboratory Findings in hemolysis

Haptoglobin Decreased as it binds free hemoglobin

Lactate Dehydrogenase Elevated as it is released from lysed cells

Peripheral Blood Smear Abnormal

Reticulocytes Increased due to bone marrow’s response to anemia and increased

RBC production
Unconjugated bilirubin Increased as it is released from lysed RBCs

Urinalysis Urobilinogen, +blood

Direct Antiglobulin Testing Antibody positive in cases of autoimmune hemolytic

Hemolytic Anemia
5. Treatment
Goals of Treatment
The goals of treating hemolytic anemia include:
1) Reducing or stopping the destruction of red blood cells
2) Increasing the red blood cell count to an acceptable level
3) Treating the underlying cause of the condition
4) Treatment will depend on the type, cause, and severity of the hemolytic anemia .
 Treatments for hemolytic anemia include :
1) Blood transfusions
2) Medicines
3) Plasmapheresis (PLAZ-meh-feh-RE-sis),
4) Surgery
5) Blood and marrow stem cell transplants
6) Lifestyle changes
Hemolytic Anemia
5. Treatment (Cont.)
1) Blood Transfusions
 Blood transfusions are used to treat severe or life-threatening hemolytic anemia.
 A blood transfusion is a common procedure in which blood is given through an intravenous (IV) line in
blood vessels. Transfusions require careful matching of donated blood with the recipient's blood.
2) Medicines
 Medicines can improve autoimmune hemolytic anemia (AIHA). Corticosteroid medicines, prednisone,
can stop immune system from making antibodies against RBCs .
 If don't respond to corticosteroids, other medicines to suppress immune system. Rituximab and
Cyclosporine.
 In severe sickle cell anemia, hydroxyurea is used .It prompts body to make fetal hemoglobin.
Hemolytic Anemia
5. Treatment (Cont.)
3) Plasmapheresis
 It is a procedure that removes antibodies from the blood. Blood is taken from body using a needle
inserted into a vein.
 The plasma, which contains the antibodies, is separated from the rest of the blood. Then, plasma from a
donor and the rest of the blood is put back in body.
 This treatment may help if other treatments for immune hemolytic anemia don't work.
4) Surgery ( Splenectomy )
 Some people who have hemolytic anemia may need surgery to remove their spleens.
 An enlarged or diseased spleen may remove more red blood cells than normal, causing anemia.
Removing the spleen can stop or reduce high rates of red blood cell destruction.
Hemolytic Anemia
5. Treatment (Cont.)
5) Blood and Marrow Stem Cell Transplant
 In some types of hemolytic anemia, thalassemia's, the bone marrow doesn't make enough healthy RBCs.
 A blood and marrow stem cell transplant replaces damaged stem cells with healthy ones from another person (a donor). During the
transplant, which is like a blood transfusion, one get donated stem cells through a tube placed in a vein. Once the stem cells are in
body, they travel to bone marrow and begin making new blood cell.
6) Lifestyle Changes
1) In AIHA with cold-reactive antibodies, try to avoid cold temperatures. This prevent the breakdown of RBCs. It's very
important to protect your fingers, toes, and ears from the cold.
 To protect , one can:
 Wear gloves or mittens when taking food out of the refrigerator or freezer.
 Wear a hat, scarf, and a coat with snug cuffs during cold weather.
 Turn down air conditioning or dress warmly while in air-conditioned spaces.
 Warm up the car before driving in cold weather.
2) People born with G6PD deficiency can avoid substances that may trigger anemia.
Hemolytic Anemia
5. Treatment (Cont.)
Treatment of hemolytic Anemia

 Treatment of Warm antibody Autoimmune Haemolysis Anaemia :

 Removal of cause
 Corticosteroid therapy
 Immunosuppressives
 Blood Transfusions
 Splenectomy
 Treatment of Cold antibody Autoimmune Haemolysis Anaemia :
 Keeping patient warm
 Immunosuppressive medication
 Treating underlying cause
Hemolytic Anemia
5. Treatment (Cont.)