Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired)...
Transcript of Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired)...
![Page 2: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/2.jpg)
Syllabus of „Anaemia 1“
1. What is anaemia?
2. The anaemic syndrom.
3. Anaemia_sign versus Anaemia_disaease
4. Why and how anaemia develops?
5. Classifications of anaemias
6. Summary of the most important items of the
lecture.
![Page 3: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/3.jpg)
Signs versus symptoms
Signs are commonly distinguished fromsymptoms as follows:
Both are something abnormal
• a symptom is experienced and reported by the patient,
while
• a sign is discovered by the physicianduring examination of the patient.
![Page 4: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/4.jpg)
1. What is anaemia?
![Page 5: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/5.jpg)
The anaemia is
pathological condition
characterized by
specific signs and symptoms.
![Page 6: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/6.jpg)
Symptoms of anaemia
• fatigue
• shortness of breath
• pale skin and muccosae
• palpitations
![Page 7: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/7.jpg)
The major criteria (signs) for
anaemia are results of
laboratory examination
• decreased number of red blood cells in the blood
• decreased concentration of hemoglobin in the blood
• a low haematocrit
![Page 8: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/8.jpg)
![Page 9: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/9.jpg)
The haemoglobin concentration in
blood should be below
• in men < 120 g Hb/L
• in women < 110 g Hb/L
![Page 10: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/10.jpg)
A life threatening anaemia
haemoglobin concentration in blood
40 to 30 g Hb/L
(an acute onset anaemia
vs. chronic anaemia)
Adaptive, compensatory mechanisms
in acute and chronic anaemia.
![Page 11: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/11.jpg)
Adaptive, compensatory mechanisms
in acute and chronic anaemia
Acute anaemia:
• increased cardiac output
• increased arterio-venous oxygen content
difference
Chronic anaemia:
• increased arterio-venous oxygen content
difference
• decreased affinity of blood to oxygen
(2,3-DPG)
![Page 12: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/12.jpg)
![Page 13: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/13.jpg)
2. The anaemic syndrome.
![Page 14: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/14.jpg)
A syndrome (generally) includes severalsymptoms occurring together - this is helpful in
the diagnostic process.
The anaemic syndrome includes:
• mucosal ev. skin pallor
• exertional dyspnea (shortness of breath)
• a rapid heart rate (tachycardia),
• fatigue,
• sleepiness
(all the symptoms depend on a degree ofanaemia and a rate of its development)
![Page 15: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/15.jpg)
3.
Anaemia_sign
versus
Anaemia_disaease
![Page 16: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/16.jpg)
Anaemia means that something is
wrong – it is
sign of a disease.
It can be a part of various diseases
(e.g. a tumour, a chronic immflamatory
disease) – „secondary anaemia“).
It can be caused by
a primary disease of the blood
forming tissues or red blood cells.
![Page 17: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/17.jpg)
• Symptomatic treatment – transfusion
of the red cell mass or the whole blood
• Causal treatment – after establishing
cause of anaemia – e.g. vitamin B12 or
iron supplementation, splenectomy,
immunosuppressive therapy ...
The symptomatic versus the causal
treatment of anaemia
![Page 18: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/18.jpg)
4. Why and how anaemia
develops?
![Page 19: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/19.jpg)
Anaemia results from
the losses of red blood cells
outweighing
their production rate.
Losses and production of red blood cells
are normally well balanced ...
![Page 20: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/20.jpg)
Control of the Red Blood Cell Production
![Page 21: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/21.jpg)
Cells producing
erythropoietin in
the kidney
according to
oxygen availability
Copyright © 2011, American Society for Microbiology
![Page 22: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/22.jpg)
Oxygen sensing mechanism governing
erythropoietin production
![Page 23: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/23.jpg)
COMPUTER SIMULATION
![Page 24: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/24.jpg)
Aneamia may improve, may
worsen, may be stable.
• A new equillibrium, a new dynamic steady-state between red blood cell losses and
production rates may result in
a chronic stable anaemia.
• A special case is
a compensated haemolytic syndrome
when both: the red blood cell losses and production are increased.
![Page 25: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/25.jpg)
5.
Classifications of anaemias
![Page 26: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/26.jpg)
Classification of anaemia
• pathogenetic
• morphologic (laboratory)
• etiologic
![Page 27: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/27.jpg)
Morphological (laboratory)
classification of anaemia
![Page 28: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/28.jpg)
Morphological (laboratory)
classification of anaemia
1. according to red blood and bone
marrow cell volume (size)
2. according to red blood cell
hemoglobinization
3. according to red blood cell shape
![Page 29: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/29.jpg)
Normocytic anaemia (MCV is 80–95 fL)
• after acute blood loss
• aplastic anaemia
• some haemolytic anaemias
Makrocytic anaemia (MCV higher than 95 fL)
• megaloblastic anaemia (folic acid or vitamin B12 deficit
• anaemia accompanying hypothyreosis, high number of
reticulocytes in the blood
Mikrocytic anaemia (MCV less than 80 fL)
• iron deficiency anaemia
• β-thalassaemia major
• hereditary spherocytosis
Morphological classification of anaemias - 1
![Page 30: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/30.jpg)
Mikrocytic (MCV < 80 fL)
hypochromic anaemia
![Page 31: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/31.jpg)
MACROCYTIC ANAEMIA (MCV > 95 fL)
anisocytosis, poikilocytosis
![Page 32: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/32.jpg)
Megaloblastic (and macrocytic) anaemia
from vitamin B12 or folic acid
deficiencies- bone marrow is not aplastic but there are few
reticulocytes in the blood
![Page 33: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/33.jpg)
Normochromic anaemia (MCHC is 300–350 g/L of
packed red blood cells)
• anaemias of various aetiologies
Hypochromic anaemia (MCHC below 300 g/L of
packed red blood cells)
• iron deficiency anaemia
• β-thalassaemia major
• pyridoxin (vitamine B6) responsive anaemia
• anaemia due to plumb (Pb) intoxication
Morphological classification of anaemias - 2
![Page 34: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/34.jpg)
Sideroblastic anaemia
![Page 35: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/35.jpg)
Pathogenetic
classification of anaemia
![Page 36: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/36.jpg)
1. Anaemias caused by
insufficient (low, decreased)
red blood cell production.
2. Anaemias caused by
increased red blood cell losses
(bleeding or hemolysis).
![Page 37: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/37.jpg)
Anaemias from decreased red
blood cell production
- reticulocytes are very low
in the blood
![Page 38: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/38.jpg)
Reticulocytes grossly indicate
activity of erythropoiesis
![Page 39: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/39.jpg)
(1)
Anaemia
from
decreased
red blood cell production
![Page 40: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/40.jpg)
Causes of decreased red blood
cell production
1. BONE MARROW FAILURE– aplastic anaemia
– myelodysplastic syndrome (MDS)
– leukaemia
– myelofibrosis
– tumour inflitration of the bone mrrow
2. DEFICIENCY OF ESSENTIAL FACTORS– iron deficiency
– folic acid or vitamine B12 deficiency
– severe protein malnutrition
3. LOW ERYTHROPOIETIN– severe damage of both kidneys
– chronic inflammation
– severe protein malnutrition
![Page 41: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/41.jpg)
NORMAL and APLASTIC BONE
MARROW
![Page 42: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/42.jpg)
Anaemia as a part of
pancytopenia
• pancytopenia means low numbers of all blood cells
• „myeloid“ (red blood cells, granulocytes, monocytes, platelets)
eventually also
• „lymphoid“ (different types of lymphocytes)
![Page 43: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/43.jpg)
Anaemia from deficiency of essential
factors (Fe, vitamin B12, folic acid ...)
• blood: mikrocytic or makrocytic anaemia, may be hypochromic
• bone marrow: contains enough hematopoietic cells (precursors of blood cells), they look abnormal (large –megaloblasts, changed nucleus-cytoplasmic ratio, little hemoglobin in the cytoplasm ...
![Page 44: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/44.jpg)
Iron deficiency anaemia(a microcytic hypochromic
anaemia)
• Iron (Fe2+) essential for heme synthesis
• erythropoiesis needs about 30 mg of iron
every day (gain from food is only 1 to 2 mg
a day). Iron is intensivelly recycled.
![Page 45: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/45.jpg)
IRON DEFICIENCY - BONE MARROW
![Page 46: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/46.jpg)
Vitamin B12 and folic acid deficiency
anaemia
• folic acid and vitamin B12 are needed for
synthesis desoxynucleotides
• cells in the bone marrow divide intensivelly and
this is hampered by deficiency of the factors
• vitamin B12 deficiency, but not that of folic acid,
may have also a neural symptoms manifested
by senitivity and motoric disorders
![Page 47: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/47.jpg)
Pernicious anaemia
• there is a deficit in vitamin B12 absorption
due to lack of the intrinsic factor produced
normally by gastric mucosa
• this used to be a deadly disease
(anaemia)
![Page 48: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/48.jpg)
Megaloblastic (and macrocytic) anaemia
from vitamin B12 or folic acid
deficiencies- bone marrow is not aplastic but there are few
reticulocytes in the blood
![Page 49: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/49.jpg)
(2)
Anaemia
from
increased red blood cell losses
![Page 50: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/50.jpg)
• chronic or acute bleeding (Fe losses!)
• haemolysis(red blood cells „live“ less than 120 days)
... reticulocytes counts are often elevated
- intravascular haemolysis
- extravascular haemolysis
![Page 51: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/51.jpg)
• a normocytic anaemia
• bone marrow increases production of red blood cells
(reticulocytes)
• 1 mL blood contains 0.6 mg iron (a daily gain of iron from
food is 1 to 2 mg)
• chronic bleeding often leads to iron deficiency and this
limits capacity of the bone marrow to replace lost red blood
cells
Acute and chronic bleeding
![Page 52: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/52.jpg)
• corpuscular (mostly hereditary)
• extracorpuscular (mostly acquired)
Hemolytic anaemias
![Page 53: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/53.jpg)
CORPUSCULAR HAEMOLYTIC ANAEMIASinborn, hereditary mostly (gene defects)
DEFECTS OF THE RED BLOOD CELL MEMBRANE
– hereditary spherocytosis, epitocytosis
– paroxysmal nocturnal haemoglobinuria (acquired)
DEFECTS OF ENERGY METABOLISM
– defects of enzymes of the pentose cycle (glucose-6-phosphate-
dehydrogenase, glutathion-reductase)
– defects of enzymes of the Embden-Mayerhof cycle (hexokinase,
2,3-difosfoglycerate-mutase, pyruvátkinase)
DEFECTS OF HAEMOGLOBIN SYNTHESIS OR HAEMOGLOBINOPATHIES
– thalasaemias
– some haemoglobinopathies (e.g. methaemoglobinaemia …)
![Page 54: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/54.jpg)
Hereditary spherocytosis, epiptocytosis –
spleen histology (splenomegaly)
![Page 55: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/55.jpg)
HEREDITARY ELIPTOCYTOSIS
A CELL MEMBRANE DEFECT (spectrin
or other proteins)
![Page 56: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/56.jpg)
Talassaemia, sickle cell
anaemia
• Talassaemia (alfa, beta
– major, minor)
• production of globin
chains is not
quantitatively matched
• clinica (phenotypic)
manifestation differs
• Sickle cell anaemia
(HbS)
• the most frequent
hemoglobinopathy
• ischemic episodes,
pain, CNS and other
organs damage
![Page 57: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/57.jpg)
Thalassaemias
Hb A (%) Hb F/A2 % Jiný Hb Symptoms
normal Hb (a healthy person) 90–98 2–3/2–3 none
talasaemia α (4 alely pro α-řetězec hemoglobinu)
- α / α α 90–98 2–3/2–3 žádné
- - / α α (- α/ - α) 90–98 2–3/2–3 žádné, hypochromní
erytrocyty/mikrocyty bez anémie
- - / - α* 60–70 2–5/2–3 30–40 H anémie (100–70 g Hb/l)
- - / - - 0 0 H/Bartův hydrops fetalis
talassaemia β (2 alely pro β-řetězec)
β0/β nebo β+/β** 90–95 2–10/5–7 žádné, mírná anémie
β + /β +*** 15–75 20–80/2–5 anémie (90–70 g Hb/l), hepato-,
splenomegalie
β0/β0**** 0 95–98/2–5 těžká anémie, poruchy vývoje,
patologické fraktury kostí, velká potřeba
transfúzí
hemoglobin Lepore („L“)
(fúze genů pro β- a δ-řetězce)
– heterozygot 70–80 5–20/1–2 5–15 „L“ mírná anémie
– homozygot 0 70–90/0 10–30 „L“ těžká anémie a jako u thalassaemia
major
* označovaná též jako „nemoc hemoglobinu H“
** thalassaemia minor
*** thalassaemia intermedia
**** thalassaemia major
![Page 58: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/58.jpg)
Talassaemia β
Hb A (%) Hb F/A2 Manifestation
β0/β nebo β+/β** 90–95 2–10/5–7 none, a mild anaemia
mírná anémie
β + /β +*** 15–75 20–80/2–5 anaemia (90–70 g
Hb/l), hepatosplenomegaly
β0/β0**** 0 95–98/2–5 severe anaemia,
transfusions, developmental
anomalies
** thalassaemia minor
*** thalassaemia intermedia
**** thalassaemia major
![Page 59: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/59.jpg)
EXTRACORPUSCULAR HAEMOLYTIC ANAEMIAS
(mostly acquired)
ANTIBODIES AGAINST RED BLOOD CELLS
– autoantibodies
– anti-Rh antibodies (e.g. fetal erythroblastosis)
– isoaglutinins
DAMAGE FROM PHYSICAL OR TOXIC FACTORS
– mechanical damage (DIC, heart valve prosthesis, march
haemoglobinuria)
– extensive burns
– bacterial toxins
– parasitic infection (malaria)
– severe plasma hypotonia
![Page 60: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/60.jpg)
Etiologic (casual) classification
of anaemias
Causes of a decreased production of red blood cells or increased red blood cell losses are numerous.
Their identification allows for a rational, etiologicaly based (causative) therapy, if possible.
![Page 61: Prezentace aplikace PowerPoint - Univerzita Karlova · •extracorpuscular (mostly acquired) Hemolytic anaemias. CORPUSCULAR HAEMOLYTIC ANAEMIAS inborn, hereditary mostly (gene defects)](https://reader033.fdocumentos.tips/reader033/viewer/2022060215/5f05b85d7e708231d4145d9f/html5/thumbnails/61.jpg)
Summary of the most important
items of the lecture.
• Anaemia may be symptom, sign or disease depending
on level of the diagnostic precedure
• Anemia results from losses of red blood cells exceeding
their production
• A new balance between losses and production of red
blood cells may result in „stable“ – chronic anaemia
• Identification of the cause of anaemia allows casual
therapy
• Transfusions of red blood cells are only a symptomatic
therapy