pathophysiology of rbc ( red blood cells)
DESCRIPTION
reb blood cellsTRANSCRIPT
content
• History of RBC• Function• Shape and size• Concentration• Production• Pathologies- polycythemia• aneamia
RED BLOOD CELL
HISTORY:- Dutch biologist Jan Swammerdam in 1658 Anton van Leeuwenhoek in 1674 In 1901, Karl Landsteiner- BLOOD GROUP
The oldest intact red blood cells ever discovered were found in Ötzi the Iceman, a natural mummy of a man who died around 3255 BC. These cells were discovered in May 2012.
FUNCTIONS
Shape and Size of Red Blood Cells
Concentration of Red Blood Cells in the Blood
Hb RBC (SI unit) USA UNITMales 13-18g/dL (4.0-5.5)x 1012/L 4.0-5.2 x
106 /mm3
Females 11.5-16.5g/dL (3.5-5.0)x 1012/L 3.5-5.0x 106 /mm3
Neonates 17-20g/dL (6.0-7.0)x 1012/L 6.0-7.0 x 106 /mm3
Erythropoiesis is the process by which the origin, development and maturation of erythrocytes occur
PRODUCTION OF RED BLOOD CELLS
IN EARLY WEEK OF EMBRYONIC LIFE- DURING SECOND TRIMESTER OF GESTATION- AFTER BIRTH- AFTER 20 YEARS:-
GENESIS of Red Blood Cells
ProerythroblastStem cell Early
erythroblast
Intermediate erythroblast Reticulocyte RBC
POLYCYTHEMIA VERA-myeloproliferative disorder-excessive proliferation of erythroid elements-RBC 6 to 12 million/cubic mm with a hemoglobin concentration of 18 to 24 g/Dl-increased blood viscosity and thrombosis-clinical picture of ruddy cyanosis is seen on the face and extremities
Clinical Manifestations
TREATMENT
Myelosuppresive drugs like hydroxyurea can be used
SECONDARY POLYCYTHEMIA: ERYTHROCYTOSIS
• increase in erythropoietin production to compensate for hypoxia
• High altitudes• Chronic pulmonary disease
APPARENT POLYCYTHEMIA
• increased hemoglobin concentration and packed-cell volume
• normal RBC mass
ANEAMIA
decrease in the normal amount of circulating hemoglobin
Anemia may be defined as reduction of hemoglobin concentration per unit volume of blood below the lower limit of normal range for age & sex of the individual
CLASSIFICATION OF ANEAMIA
Common symptoms
With dry mouth
1: Clinical features:-
What can be the cause??
Complete blood picture• cells are microcytic and hypochromic• hemoglobin value is less • low serum iron concentrations • ferritin levels are markedly reduced.• high serum iron-binding capacity• absence of stainable iron in the bone marrow
• The physician must perform a thorough search for the source of bleeding, including using radiologic surveys of the gastrointestinal tract, sigmoidoscopy, a gynecologic examination, and a complete menstrual and dietary history.
diagnosis
DAILY REQUIREMENT OF IRON
treatment• -ferrous sulphate 200 mg three times daily• -Alternative preparations include ferrous
gluconate and ferrous fumarate.• -Iron sorbitol injection is a complex of iron, • -sorbitol and citric acid• 10-20 deep intramuscular injections are given
over two to three weeks.
Clinical features
- ATROPIC ORAL MUCOSA
- ATROPIC MUCOSA OF PHARYNX , UPPER ESOPHAGUS.
- SPASM IN THROAT
- FOOD STICKING IN THROAT
- DYSPHAGIA
• First described by Plummer and Vinson• Plummer-Vinson syndrome is potentially
serious because pharyngeal and intraoral carcinoma are more common in these patients.
• Patients with symptoms of this syndrome should be followed up at short intervals and checked for the development of lesions that raise the suspicion of malignancy.
treatment
• IRON THERAPY HELPS
HEMOLYTIC ANEAMIA
extracorpusc
ular
intracorpuscular
diagnosis• -decreased hemoglobin, increased reticulocytes.• Coombs test directLooks for immunoglobulin &/or complement of
surface of red blood cell (normally neither found on RBC surface)• Coombs reagent - combination of anti-human immunoglobulin & anti-human
complement• Mixed with patient’s red cells; if immunoglobulin or complement are on surface,
Coombs reagent will link cells together and cause agglutination of RBCs• INDIRECT coomb• Looks for anti-red blood cell antibodies in the patient’s serum, using a panel of
red cells with known surface antigens• Combine patient’s serum with cells from a panel of RBC’s with known antigens• Add Coombs’ reagent to this mixture• If anti-RBC antigens are in serum, agglutination occurs
Clinical features
• pallor of nails bed, sclera , skin , soft palate , tongue .
• Produces jaundice due to hyperbilirubineamia
Hemolysis due to:-
Paroxysmal nocturnal hemoglobinuriadefect is an acquired clonal stem cell disorder that results in abnormal sensitivity of the RBC membrane to lysis by complement.
Glucose 6 phosphate dehydrogenase deficiency
Lack of G6PD leads to hemolysis during oxidative stress
• Due to hemolysis hb can be reduced by 3-4g/dl
• Drugs that induce hemolysis should be avoided such as dapsone,phenacetin.
c/f
Glossitis , Glossodynia
red beefy tongue
Dysphagia and taste change
Burning mouth
Dysphagia , taste aberrations
c/f
cause
Lab finding
macrocytic normochromic red cells MCV increasedMCH increasedshape of the red cells variespresence of megaloblastic marrow changesSchilling test is used
Causes of B12 deficiency
42
B12 deficiency
Inadequate intake
Increased need
Impaired absorption
Lack of IF
Malabsorption
Gastrectomy
Perniciousanaemia
treatment
administration of parenteral cyanocobalamin1000 μg/day for 1 week, then 1000 μg/week for 1 month, followed by 1000 μg/month for life
Clinical feature
Lab findings
• low serum assays of folic acid• macrocytic normochromic red cells • MCV increased• MCH increased• shape of the red cells varies• presence of megaloblastic marrow changes
Treatment
5mg per day for 3 weeks sufficient
c/f
Absent or hypoplastic thumbMental and sexual retardation
Causes of aplastic
Aplastic aneamia
• Caused by bone marrow failure.
• Fanconi aneamia:- IS INHERITED APLASTIC ANEAMIA
Lab findings
• Sickle shaped cell• Hemoglobin electrophoresis is less expensive,
more accurate, and more definitive in the diagnosis of sickle cell disease as it detects hemoglobin S.
• chipmunk facies.”• Frontal bossing, maxillary hypertrophy,
depression of nasal bridge , Malocclusion of teeth
PARAVERTEBRAL MASSES:• Broad expansion of ribs at vertebral
attachmentPATHOLOGICAL FRACTURES:• Cortical thinning• Increased porosity of long bones DELAYED PNEUMATISATION OF SINUSES
X ray skull:
“ hair on end” appearance
or“crew-cut” appearance
• The skin color becomes ashen-gray due to the combination of pallor, jaundice, and hemosiderosis.
• Patients also present with cardiomegaly, hepatomegaly, and splenomegaly
Lab findings• Hemolytic anemia with hypochromic microcytic• red blood cells that vary in size and shape • increased amounts of fetal hemoglobin
• Prenatal diagnosis of thalassemia is facilitated by deoxyribonucleic acid(DNA) analysis of amniotic fluid cells, and it plays an important role in genetic counseling.
References:--DAVIDSONS- GENERAL MEDICINE 20TH EDITION-ABC OF CLINICAL HAEMATOLOGY- 2ND EDITION-GUYTON – TEXT BOOK OF MEDICAL PHYSIOLOGY 11TH EDITION-WILLIAMS HEAMATOLOGY- 7TH EDITION-BURKETS – TEXT BOOK OF ORAL MEDICINE – 7TH EDITION
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• BY DR.CHINTAN SAVANI
PATHOPHYSIOLOGY OF WHITE BLOOD CELLS