hemolytic & aplastic anemia

1. Hemolytic & aplastic anemias

2.  Definition:
 Those anemias which result from an increase in RBC
destruction coupled with increased erythropoiesis
 Classification:
 Congenital / Hereditary
 Acquired

3. INTRACORPUSCULAR
DEFECTS
EXTRACORPUSCULAR
FACTORS
HEREDITARY •HEMOGLOBINOPATHIES
•ENZYMOPATHIES
•MEMBRANE-
CYTOSKELETAL DEFECTS
•FAMILIAL HEMOLYTIC
UREMIC SYNDROME
ACQUIRED •PAROXYSMAL
NOCTURNAL
HEMOGLOBINURIA
•MECHANICAL
DESTRUCTION
[MICROANGIOPATHIC]
•TOXIC AGENTS
•DRUGS
•INFECTIOUS
•AUTOIMMUNE

4.  DEFINATION:-
this is an inhereted form of
hemolytic anemia,which is based on a defect
membrane proteins erythrocytes that leads to
disruption of membrane permiability,entry of
excess water,where by the red blood cells become
rounded shape with their subsequent destruction
in the spleen
ETIOLOGY;-
congenital pronounced ertrocyte
membrane defect,inhereted in an autosomal
dominant type.

5.  GENERAL EXAMINATION - JAUNDICE, PALLOR
BOSSING OF SKULL
 PHYSICAL FINDINGS - ENLARGED SPLEEN
 HEMOGLOBIN - FROM NORMAL TO SEVERELY REDUCED
 MCV - USUALLY INCREASED
 RETICULOCYTES - INCREASED
 BILIRUBIN - INCREASED[MOSTLY UNCONJUGATED]
 LDH - INCREASED
 HAPTOGLOBULIN - REDUCED TO ABSENT

6.  Deformation of skeleton and the skull:the
square shape of the head “the small size of the
orbit of the eye
 Changes in the location of teeth
 Fusion of fingers together
 Increasing the no of fingers on both hands and
feet
 splenomegaly

7.  This hemolytic anemia,caused by the formation of
antibiotics in the peripheral blood for their own
red blood cells,leading to their destruction.
 ETIOLOGY:-
this etiology is not fully known.

8.  Specific features of anemic syndrome
 Specific features of hemolytic anemiea
 This disease is often compared to other
autoimmune diseases
 LAB DIAGNOSIS:-
antierythrocyte appearance of
antibodies in the blood

9.  Hemoglobin electrophoresis
-HbS >80%
-HbF -1-20%
-HbA2 -2- 4.5%
 Sickling test POSITIVE

10.  Hemo.anemia,reticulocytosis,granulocytosis
 Vasoocclusion-protean
 Painful crises
 Splenic sequestration crises
 Hand foot syndrome
 Acute chest syndrome

11. Condition Clinical
abnorm
Hb level g% MCV,fl Hb electropho
Sickle cell trait None,rare
painlss
hematuria
normal normal HbS/A:
40/60
Sickle cell
anemia
Vasocclusive
crises,AVN,gal
lstones,
priapism
7-10 80-100 HbS/A:100/0
HbF;2-25%
S/beta0
thalasssemia
Vasoocclusive
Crises,AVN
7-10 60-80 HbS/A-100/0
HbF; 1-10%
S/beta+
thalassemia
Rare crises,
AVN
10-14 70-80 HbS/A:
60/40
HbSC --do--,
retinopathy
10-14 80-100 HbS/A;50/0
HbC;50%

12. Heriditary:-
main and mosf effective treatment is
splenectomy,leading to clinical recovery.
Acquired:-
inhibition of the formation of antibodies to eretrocytes
 Splenectomy
 Immunosuppressive therapy
 Plasma exchange
 RBC transfusion

13.  Defination:-
aplastic anemia is a rare disease in
which the bone marrow and the hematopoietic
stem cells that reside there are damaged. This
causes a deficiency of all three blood cell types
(pancytopenia): red blood cells (anemia), white
bloodcells (leukopenia),
and platelets (thrombocytopenia).Aplastic refers
to inability of the stem cells to generate mature
blood cells

14.  In half cases its unknown etiology others may
include
 Chemical factors:-
benzene,leaded gasoline,pesticydes
 Physical factors:-
ionizing radiation,x-rays
 Medications:-
chlormphenicol,sulfonamides,nsaids and other
cyto toxic agents

15.  Nasal,uterine,gastrointestinal,and renal
bleedings
 Bleeding gums
 Punchulate rash on skin and mucous
membrane
 Hemorrhage in the brain,retina and sclera
 Infectious complicants:-
abrasions,infiltrates or abscesses at the
injection site,pneumonia,sepsis etc.

16.  a complete blood count, renal
function and electrolytes, liver
enzymes, thyroid function tests, vitamin B12 and folic
acid levels.
 Bone marrow aspirate and biopsy: to rule out other
causes of pancytopenia
 History of iatrogenic exposure
to cytotoxic chemotherapy: can cause transient bone
marrow suppression

17. Instrumental diagnosis :-
X-rays, computed tomography (CT)
scans, or ultrasound imaging tests: enlarged lymph nodes
(sign of lymphoma), kidneys and bones in arms and hands
Chest X-ray: infections
Liver tests: liver diseases
Viral studies: viral infections
Vitamin B12 and folate levels: vitamin deficiency
Blood tests for paroxysmal nocturnal
hemoglobinuria
Test for antibodies: immune competency

18.  treating immune-mediated aplastic anemia
involves suppression of the immune system, an
effect achieved by daily medicine intake, or, in
more severe cases, a bone marrow transplant.
 Cytotoxic agents.
 Corticosteroids in auto immune form.
 Splenctomy
 Transfusion of blood products.RBCs,pletelets
etc.