Hemolytic Anemias

Question 1

Atypical hemolytic uremic syndrome (aHUS)

*aHUS →mutations in any one of several genes encoding complement regulatory proteins

Answer 1

Is an extremely rare disease
(hemolytic anemia),
low platelet count (thrombocytopenia)
inability of the kidneys to process waste products from the blood and excrete.(50% of cases progressing to end-stage renal disease (ESRD))

treatment has been plasma exchange
Anti C5 complement inhibitor(Eculizumab)./abrogate need of plasma exchange.
relapses are always possible , continuing eculizumab,especially in order to prevent ESRD.

HUS] caused by infection with Escherichia coli producing the Shiga toxin, regarded as typical.

Question 2

General and clinical abnormalities of hemolytic anemias

Answer 2

Jaundice
Urine discolouration
Splenomegaly (some cases hepatomegaly)

Question 3

Extravascular hemolytic anemia

Answer 3

In liver and spleen (reticuloendothelial sys).

Question 4

Intravascular hemolysis
خوين كةفدة مييز

Answer 4

Hemoglobinuria

(often associated with hemosiderinuria); in the serum there is free hemoglobin,
LDH is increased,
haptoglobin is reduced
In contrast, the serum bilirubin level may be normal or only mildly elevated.

The main sign of the erythropoietic response by the BM → ↑ Retic
The increased number of reticulocytes is associated with an increased MCV in the blood count.

PBS → macrocytes; polychromasia, and sometimes one sees NRBC

In most cases, a bone marrow aspirate is not necessary in the diagnostic workup; if it is done, it will show erythroid hyperplasia.

Question 5

Hemolytic anemia:-

Intravascular

Extravascular

Answer 5

hemoglobinuria (often associated with hemosiderinuria)/dec of serum hemoglobin /هيموگلوبين وة ئازايي/ in the serum there is free hemoglobin, LDH (lactate dehydrogenase) is increased, and haptoglobin is reduced.
In contrast, the serum bilirubin level may be normal or only mildly elevated.

In reticuloendothelial sys — زياتر تيكشكان—Incof unconjugated bilirubin /Inc of AST (liver) /urobilinogen will be increased in both urine and stool.

In both forms of hemolysis, there is anemia and jaundice.
Hemoglobinemia and hemoglobinuria occur only in intravascular hemolysis.
Hypertrophy of the mononuclear phagocyte system and consequent splenomegaly are seen only in extravascular hemolysis.

Question 6

In hemolytic anemia MCV is increased due to

Answer 6

Inc of reticulocyte in blood count

Question 7

What can be seen in
peripheral blood smear PBS

BM aspiration

Answer 7

macrocytes;
polychromasia,
sometimes NRBC(nucleated)

Erythroid hyperplasia

Question 8

Hereditary spherocytosis

Answer 8

Can be Autosomal dominant or recessive.

Question 9

In hereditary spherocytosis
Inc of MCHC

Answer 9

Microcytosis with Inc of MCHC /often normocytic

طوپ بویچگ بویچگ پر له تراکیز هیموگلوبین

Inc of RDW

Question 10

Clinical features of hereditary spherocytosis

Answer 10

Sever in infancy / mild in young or latter in life

jaundice,
enlarged spleen,
gallstones(it may be the finding of gallstones in a young person that triggers diagnostic investigations)

Question 11

Treatment of hereditary spherocytosis

Before splenectomy,

Answer 11

vaccination against encapsulated bacteria (Neisseria meningitidis and Streptococcus pneumonia) is imperative

Question 12

Dehydrated stomatocytosis
کأنما دةم ديرد

Answer 12

Dehydrated hereditary stomatocytosis (DHS) is a rare hemolytic anemia
characterized by a decreased red cell osmotic fragility due to a defect in cation permeability, resulting in red cell dehydration and mild to moderate compensated hemolysis

Question 13

Stomatocytosis:-

1/ Dehydrated DHS
2/Overhydrated OHS

Answer 13

The erythrocytes’ cell membranes may abnormally ‘leak’ sodium and/or potassium ions, causing abnormalities in cell volume

1/dehydrated (xerocytosis) /leak of potassium ions /dec of MCV 👇/Inc of MCHC👆/compensated hemolysis/mild jaundice.
2/overhydrated (hydrocytosis) /MCV👆/MCHC👇.

Question 14

قصةي G6PD Deficiency

Answer 14

هذا الأنزيم يصنع غلوتاثيون الذي يحمي الخلايا الحمراء من العوامل المؤكسدة ،
ولك ان تتخيل ماذا يحدث عند نقصه(اليرقان وانحلال الدم)🤗

Persistent neonatal jaundice /often associated with reticulocytosis/ is usually normo-macrocytic.

Bil and LDH are increased.

hemolysis is,chronic in these patients, they are also vulnerable to acute oxidative damage.

Mainly intravascular hemolysis ,but extravascular also can occur (unconjugated hyper-bilirubinemia).

Question 15

G6PD deficiency has been selected by……………. malaria because it confers a relative resistance against this highly lethal infection

Answer 15

Plasmodium falciparum

Question 16

splenectomy in G6PD deficiency cause

Answer 16

increase in hemoglobin (paradoxically, often reticulocytes also increase considerably).

Question 17

Treatment of G6PD deficiency

Answer 17

Supportive /folic acid suplemention.
Blood transfusion
Iron chelation

In sever —BMT

Question 18

Drugs that may develop G6PD deficiency attack

Answer 18

سلايد ١٥

Question 19

Because hemolysis in G6PD Deficiency occurs intravascularly

Answer 19

hence, it is associated with
hemoglobinemia,
hemoglobinuria,
high LDH,
low or absent plasma haptoglobin.

Question 20

PBS In G6PD deficiency

Answer 20

Anisocytosis
Polychromasia
Spherocytes
Bizarre poikilocytosis
Hemighost
Bite or blister cells

Question 21

In G6PD deficiency blood film …………..

Answer 21

Heinz bodies

Question 22

The most serious threat from AHA (Acute hemolytic anemia ) in adults is

Answer 22

the development of acute renal failure (this is exceedingly rare in children).

Question 23

A very small minority of subjects with G6PD deficiency have CNSHA
(Chronic nonspherocytic hemolytic anemia) .

Answer 23

nearly always a male,
usually with a history of NNJ
who may present with anemia, unexplained jaundice,
or gallstones later in life.
The spleen may be enlarged.

Question 24

Labortary diagnosis of G6PD deficiency

Answer 24

oldest, most G6PD-deficient red cells have been selectively destroyed, and young red cells, having higher G6PD activity, are being released into the circulation.

Only quantitative test , can give a definitive result .

Question 25

PYRIMIDINE 5’-NUCLEOTIDASE (P5N) DEFICIENCY

Answer 25

Highly distinctive feature of this condition → basophilic stippling

Question 26

Auto autoimmune haemolytic anemia

Answer 26

onset → often abrupt and can be dramatic→ The Hb may drop, within days, to as low as 4 g/dL; the massive red cell removal will produce jaundice, and sometimes the spleen is enlarged

reticulocyte count is typically elevated
LDH may also be elevated.

Question 27

sometimes……….. may be the first manifestation that leads to a diagnosis of SLE or lymphoproliferative disorders

Answer 27

WAIHA

Question 28

When severe anemia is associated with reticulocytopenia, the use of……………. may help to reduce or avoid the requirement for transfusion of red cells

Answer 28

erythropoietin

Question 29

A full budd chiari syndrome without liver disease

Answer 29

Paroxysmal nocturnal Hemoglobinuria

Question 30

PNH is a normo-macrocytic anemia

But can become microcytic due to

Answer 30

Chronic hemolysis hence iron deficiency

Question 31

PNH can result in

Answer 31

Unconjugated bilirubin is mildly or moderately elevated
LDH is typically markedly elevated
Haptoglobin is usually undetectable.