This document discusses several types of hemolytic anemias including glucose-6-phosphate dehydrogenase enzyme deficiency, proximal nocturnal hemoglobinuria, autoimmune hemolytic anemia, and malaria. It covers the pathophysiology, symptoms, diagnosis, treatment and prognosis of each condition. Key details provided include common causative agents, effects on red blood cells, diagnostic tests used, and types of treatment options available.

1. Kalsoom Saleem
CMS # 8107

2. Glucose-6-Phosphate Dehydrogenase Enzyme
Deficiency
• Pathophysiology

3. Glucose-6-Phosphate Dehydrogenase Enzyme
Deficiency

4. Causative Agents
• Food-Fava beans
• Medications-Sulfasalazine
Reduced Glutathione
• Consumed during oxidative
stress
Attack RBCs
• Globin chain-denaturing
hemoglobin
Heinz Bodies
• Precipitation of hemoglobin-
intracellular inclusions
Intravascular Hemolysis Extravascular Hemolysis
• Phagocytosis of less
damaged RBCs
Bite Cells
• Plucked out by phagocytes

5. Morphology
Microscopic
-Red Cells with
precipitates of
denatured globin
(Heinz bodies)
-Bite shape of cells
(Bite Cells)

6. Morphology
Gross
Yellow
coloration of
cornea due to
hemolysis of
RBCs causing
jaundice

7. Etiology
Drugs (Sulfonamides,
Ciprofloxacin, Aspirin,
Sulfonylureas)
Genetic
Kidney Failure
Diabetes
Food (Fava Beans)

8. Symptoms
Rapid heart rate
Fast breathing
Splenomegaly
Pale Skin
Elevated Body
Temperature
Dark to yellow-
orange urine

9. Complete Blood Count
and Reticulocyte Count
Heptaglobin Test
Renal Function Test
Diagnosis

10. Avoiding specific
medications, foods
and environmental
exposures
Dialysis-in kidney
failure
Supplements
Blood transfusion
Treatment

11. Prognosis
• Patients with G6PD enzyme deficiency get fewer illnesses as
compared to other people and the possible outcome is general
recovery from disease.

12. Proxymal Nocturnal Hemoglobinuria
Pathogenesis

13. Morphology
Microscopic
•Hypercellular marrow with
erythroid hyperplasia.

14. Morphology
Gross
Flushing of skin due to
pancytonpenia

15. Etiology
•Somatic mutation
in PIGA gene in
stem cells (bone
marrow)

16. Symptoms
Abdominal pain
Headaches
Back pain
Weakness/Fatigue
Splenomegaly
Recurrent infections

17. Diagnosis
Flow
Cytometry
(detection of
mutant gene,
percentage
and degree
of deficient
RBCs)
Bone Marrow
Analysis
Acid
Hemolysis
Test

18. •Corticosteroids
•Iron
Replacement
Therapy
Treatment
•Splenectomy
•Folate
Supplements
Treatment Stem Cell
TransplantationTreatment
•Complement
Inhibitors
(Ecluzimab)
•Blood
Transfusion
Treatment

19. Prognosis
Death results after 10-11 years of diagnosis with
decreased cell number

20. Autoimmune Hemolytic Anemia
• Some people develop antibodies -interact with determinants on RBCs - causing
hemolytic anemia.
• Classified according to
• nature of the antibody
• presence of predisposing conditions.
• Warm Antibody Immunohemolytic Anemia:
• It is caused by the IgG or IgA (rarely) immunoglobulins which are active at room
temperature (370C) that attach to RBCs membranes making their Fc portion
exposed which get attached to Fc receptors on monocytes and macrophages. This
will allow them to phagocytize the RBC membranes changing them to
spherocytes. Spherocytes are not flexible as normal RBCs and are, therefore,
erythrophagocytosed in Spleen leading to hemolysis.

21. Autoimmune Hemolytic Anemia
• Cold Antibody Immunohemolytic Anemia:
• Circulating antibodies esp. IgM causes the destruction of RBCs
• Their concentration in normal being is too low to trigger disease
• Patients with CAIA have higher concentrations of IgM
• At low temperatures i.e. below 300C, IgM antibodies bind to glycoproteins on the
surface of RBCs activating the complement system which then damages the RBCs
by forming Membrane Attack Complex injecting proteins into the RBCs forming
pores leading to membrane instability causing intravascular hemolysis. If the
complement response is insufficient, then extravascular hemolysis occurs due to
deposition of MAC proteins viz. C3b and C4b on RBCs activating opsonization
process and their destruction by phagocytosis in spleen.

22. Idiopathic
HIV infection
autoimmune diseases
(e.g.systemic lupus
erythematosus (SLE)
lymphoproliferative
disorders (e.g. non-
Hodgkin's lymphoma)
lymphoproliferative
disorders
Mycoplasma
pneumonia
Infections such as
Epstein-Barr virus
Idiopathic
Etiology
Warm AIHA Cold AIHA

23. Fatigue
Fever
Splenomegaly
Symptoms
Symptoms
Breathing
difficulty
Symptoms
Jundice
Dark Urine
Chest Pain

24. Diagnosis

25. Treatment
Blood Transfusion
Corticosteroids
Immunosuppressants
Plasmapheresis
Bone Marrow
Transplant
Life style changes

26. Malaria
Pathogenesis

27. Morphology
Microscopic
• Blood smear showing red blood cells that contain developing P.
vivax parasites

28. Etiology
Female
Anopheles
Mosquito
Infected
blood
through
Blood
Transfusion
Organ
Transplant

29. Symptoms
Abdominal
pain
Poor appetite
Muscle aches
Orthostatic
hypotension
High fevers
Headache
Chills and
sweats
Diarrhea,
nausea, and
vomiting

30. Blood Test
Serological
Testing
Diagnosis

31. Treatment
Quinine
followed by
doxycycline or
clindamycin
Chloroquine
tablets (eg
avloclor,
malarivon or
nivaquine)
Atovaquone
with proguanil
(malarone)
Artemether
with
lumefantrine
(riamet)