RBC Disorders Flashcards
1) Source of erythropoietin
2) Formation of granulocytes and monocytes
3) Commonest site bone marrow taken
4) Distinguish b/w IDA & Thalessemia trait
5) Formation of platelets require
6) Drug given to anti cancer patient in anemia
7) Recombinant GM-CSF & G-CSF
8) Recombinant IL-11
1) Peritubular cells of kidney
2) GM-CSF & G-CSF
3) In adults - post sup iliac spine
In child - Tibia
4) Mentzer index for IDA >13 & Thalessemia <13
5) IL-11
6) Darbopoeitin
7) Sargramostin & Filgrastim
8) Oprelvekin
1) Formation of platelets require
2) Drug given to anti cancer patient in anemia
3) Recombinant GM-CSF & G-CSF
4) Recombinant IL-11
5) RDW
6) Anemia diagnosis criteria
1) IL-11
2) Darbopoeitin
3) Sargramostin & Filgrastim
4) Oprelvekin
5) Variation in size of RBCs (anisocytosis)
11.5-14.5
6) Male -13 g/dl
Female - 12 g/dl
Pregnant female - 11 g/dl
1) First cell having appearance of Hb
2) Routine staining appearance of Hb
3) Last stage in development of nucleated RBC
4) Reticulocyte Normal range & stain
5) Reticulocyte is called
6) Maintains shape of RBC
7) MCV
8) MCH
9) MCHC
1) Erythroblast
2) Intermediate normoblast
3) Late normoblast
4) 0.5-2% & supravital stain - new methylene blue
5) Poorman bone marrow aspirate
6) Spectrin
7) 80-100 femtolitres
8) Hb inside RBC 27-32 picograms
9) 34-37 g/dl ; <34 - anemia
Examples of microcytic RBCs
<80 FL Sideroblastic anemia Iron deficiency anemia (MC) Thalessemia Anemia of chronic disease Lead poisoning
Examples of macrocytic RBCs
>100 FL Liver disease Hypothyroidism Megaloblast (B12, folate def.; Methotrexate) Cell changes
Findings in hemolytic anemia
Anemia Serum LDH inc Unconjugated bilirubin & jaundice Free Hb (toxic) release attach to Haptoglobin Dec. in haptoglobin Hemoglobinemia & hemoglobinuria
Hemoglobinuria causes
Altered color of urine Acute tubular necrosis Renal hemosiderosis Inc. in bone marrow activity causes decrease in M:E ratio Reticulocytosis
Myeloid: Erythroid ratio
Normal 3:1
CHRONIC hemolytic anemia causes
Unconjuagted bilirubin + Calcium salts = pigment gallstones
Jaundice + anemia + alteration in urine color
Jaundice + anemia + alteration in urine color + heaptosplenomegaly
Intravaacular hemolytic anemia
Extravascular hemolytic anemia
Intracorpuscular hemolytic anemia
Extracorpuscular hemolytic anemia
Inherited - Hereditary Spherocytosis, G6PD def., Thalessemia, sickle cell anemia
Acquired - PNH
Non immune - Clostridium infection , lead poisoning
1) Which form of iron is utilised for absorption?
2) Receptor
3) Site of absorption
4) Transporter on basal surface responsible for bringing iron inside body
5) Factors responsible for iron absorption
6) Factors causing IDA
1) Ferrous form Fe2+
2) DMT -1
3) Duodenum
4) Ferroportin
5) Vit C as Ascorbic acid, HCL,Amino acids , sugar
6) Chronic disease iron deficiency renal disease
Factors decreasing iron absorption
Phytate present in Vegetables
Carbonate
Tetracycline **
1) Plasma protein transporting iron inside blood
2) 1 molecule of this protein transports how many iron molecules
3) TIBC
4) % saturation Tf
5) Serum iron
1) Transferrin
2) 2
3) 360 mg/dl
4) 33%
5) 120 mg/dl
1) Iron storage form
2) This is getting converted into
3) Calculate 2) level with ____ stain
1) Ferritin
2) Hemosiderin
3) Prussian blue called Pearls reaction
1) Iron deficiency
2) Changes in blood picture - iron profile changes
3) Gold standard for diagnosis
4) Blood test shows
1) Koilonychia, pica, pallor, dyspnea, palpitations
2) serum ferritin dec.
Transferrin saturation dec.
Serum iron dec.
TIBC inc.*****
3) Bone marrow examination
4) dec. Hb, MCV MCH MCHC all dec.
RDW inc. (pencil shaped RBC)
Target cell
Microcytic hypochromic anemia
Anisocytosis
Poikilocytosis
Free protoporphrin inc.
Most sensitive indicator of IDA
Rx for children
Rx for adult
Serum ferritin
Antihelminthic like Albendazole
Fe supplement like ferrous sulphate Fe2+
When parenteral iron is given
Oral iron is not tolerated
Iron dextran & iron sorbitol citrate
Given by ‘Z’ tract technique
1) Anemia of chronic dis which interleukin secreted & its action on bone marrow & liver
2) Rx
1) IL-6 ==> dec. erythropoietin
Normocytic normochromic anemia
serum ferritin inc.**
Transferrin saturation dec.
Serum iron dec.
TIBC dec.
Hepcidin - Inhibit metabolism of iron
Causes microcytic hypochromic anemia
2) Treat primary cause
1) Vit B12 is also called
2) Vit B12 absorbed in
3) Stomach secretes
4) What causes B12 & IF attachment
1) Cyanacobalmin containing cobalt
2) Absorbed in ileum****
3) Pepsin & Intrinsic factor
4) Pancreatic enzymes
Causes of dec. absorption of B12
Vegans and alcoholics Surgery Pernicious anemia (Oral B12 not efficient ) Pancreatic insufficiency Bacterial overgrowth syndrome Iliacdisease
Reaction of 1 carbon transfer
B12 and FA
Homocysteine——-> Methionine
DNA synthesis B12
Increased homocysteine
Thrombosis
Atherosclerosis
B12 reaction only
Methylmalonyl CoA—–> Succinyl CoA required for myelin
Neurological deficits
Increased methylmalonyl
Methylmaloniemia
Methylmaloneuria
Bone marrow studies in B12 deficiency
Pancytopenia (ineffective erythropoeisis)
RBCs : Macrocytic = Megaloblast, oval shape= Macrovaloblasts
Howell Jolly bodies, Cabot rings
WBCs : Hypersegmented neutrophils
Platelets : Abnormal megakaryocytes
GIT findings in B12 deficiency
Neurological symptoms
Mucosal atrophy
Tongue : smooth appearance
Paraesthesia Tingling sensation CNS : spinal cord involvement Dorsal column more commonly affected Sub Acute Combined Degeneration = SACD
Blood studies shows what in B12 deficiency
Hb dec.
MCV inc.
MCHC NORMAL****
Schilling test
Not useful for diagnosis of B12
Dons for differentiating causes of absorption
Infection causing B12 deficiency
Fish tapeworm = Diphyllobothrum latum
Pernicious anemia
Treatment
Autoantibodies + auto reactive T-cell against parietal cells
Dec. IF
Type 2 Hypersensitivity reaction
Cause intestinal metaplasia in fundus of stomach
Parenteral B12 (IM)
B12 parenteral is given in
Pernicious anemia
CYANIDE POISONING *****
Increase in MCHC occurs only in
Hereditary spherocytosis
Sickle cell trait relation with falciparum
Protective
Any kind of Hbpathy & G6PD deficiency is protective
1) Folate is absorbed in
2) Most active form of folic acid
3) Diagnosis
1) Jejunum
2) Methyl THF—–> THF
which is useful for
Methylene THF —-> DHF forms dTMP useful for DNA synthesis
3) RBC folate levels which do not fluctuate
Folate trap
Due to B12 deficiency in form of Methyl THF
Causes of secondary folate deficiency
Alcohol
Phenytoin
Methotrexate inhibits DHF reductase
DHF reductase DHF------------------>THF
Megaloblastic anemia but no neurological symptoms
Folate deficiency
Histidine metabolism
FA
Histidine —-> FiGLU——-> Glutamate
FiGLU in urine
Folate in pregnancy
Prior to conception so as to avoid neural tube defects such as spina bifida
Hereditary spherocytosis
Rx
AD Ankyrin is defective Jaundice Anemia Splenomegaly Anisocytosis & hyperchromic spherocytes Microspherocytes Loss of central pallor of RBC
MCV dec.
MCH Normal
MCHC inc.
Elective splenectomy
Higher chance of infection- capsulated org.
Grave of RBC
CORDS OF BILLROTH
Spleen
Spherical RBCs seen in
Autoimmune hemolytic anemia and not spherocytosis
Osmotic fragility test also called as
Pink test
RBCs placed in Hypotonic solution
Increased in spherocytosis
Decreased in sickle cell disease
Increased bone marrow activity in spherocytosis
Increased erythroid precursor
G6PD deficiency
G6PD Mediterranean
X LR
Self limiting hemolysis
Prevents oxidative damage to RBC
G6PD Glucose 6 PO4----> 6 phospogluconate
Forms NADPH—-> Reduced glutathione
In Mediterranean form, Blister cells seen
Coombs negative
Oxidative stress
Hb denaturation —> HEINZ BODY (intracellular inclusion)
Crystal violet stain
Intravascular & extravascular hemolysis
BITE CELLS in peripheral smear
BLISTER CELLS
Etilogy of oxidative stress
Fava beans
Infections
SULPHONAMIDES NITROFURANTOIN
ANTI MALARIAL DRUGS (primaquine)
Sickle cell anemia
AR
Point mutation at 6 beta globin chain
Valine instead of GLUTAMIC ACID
Polymerisation of deoxygenated Hb
BetaS
Hypoxia & dehydration
RBC sticky & trapped into spleen
Sickle cell trait & disease —HbS
Extravascular hemolytic anemia
Irreversible sickling
Micro vascular occlusion
Bones : Dactylitis ; Hand foot syndrome, avascular necrosis of femur **
Vertebral column : cord fish vertebra
H shaped vertebra , fish mouth vertebra
Skull: Crew cut appearance= hair on end
Osteomyelitis : Salmonella & staph aureus
Skin : non healing ulcer in lower limb
Penis : Priapism
Lungs : acute chest syndrome
Kidneys : papillary necrosis also in DM, hyposthenuria
Sickle cell anemia changes in spleen
Spleen :
Early : Congestive splenomegaly
GANDY GAMMA BODY
Late : Autosplenectomy
ESR in sickle cell
Dec. ESR
Dithionite metabisulfite removes O2
Distinguish sickle cell trait & disease
HPLC gives % of HbS & HbA
Hb electrophoresis
Complications in sickle cell
Vaso-occlusion crisis
Sequestration crisis
Drug in sickle cell
Hydroxyurea
Inc. HbF
Used as anti cancer drug
Factors responsible for sickling
HbS
Dehydration & deoxygenation
Acidosis
Infections
Intake of primaquine acts as a trigger for hemolysis in
G6PD deficiency
Banana like finding in smear
Plasmodium falciparum causing malaria
Hereditary spherocytosis
Due to chronic hemolysis, pigmented stones are formed in gallbladder
Howell Jolly bodies seen
Hereditary elliptocytosis
Polymerisation of spectrum molecules
Spherocytosis is seen in
Hereditary spherocytosis Autoimmune hemolytic anemia Hemolytic disease of newborn Post transfusion Heinz body hemolytic anemia (G6PD def.) Water dilution hemolysis Fragmentation hemolysis (burns, mechanical trauma)
Gene deletion
Gene mutation
Alpha Thalessemia Chr.16
4 genes
Beta Thalessemia (MC) Chr.11 2 genes
Beta zero beta zero
AR
Thalessemia major
Transfusion dependent Thalessemia
Iron overload
Alpha chain precipitation
Direct damage to normoblasts
Extravascular hemolysis
Causing anemia
Jaundice hepatosplenomegaly
Thalessemia features
Extra medullary hematopoeisis due to increased erythropoietin
Chimpanzeee chipmunk like fancies
Microcytic hypochromic anemia
Target cells
Basophilic stippling
HLPC shows :
HbF elevated in major
HbA2 elevated in minor >3.5 g/dl
Test for BETA Thalessemia
Nestrof test —> screening test
HbA2 inc.
ALPHA Thalessemia
Beta 4 tetramer = HbH
Has too much affinity for O2
Hypoxia
Fetal : Gamma 4 tetramer = Barts Hb
Hydrops fetalis
Autoimmune hemolytic anemia
Type 2 hypersensitivity
Direct Coombs test
Warm : IgG»_space; IgA
Idiopathic, SLE, rheumatoid arthritis, CLL, ALPHA METHYLDOPA
Cold : IgM»_space; IgG
INFECTIOUS MONONUCLEOSIS, Mycoplasma , malgnancies (Walden storm macroglobulinemia)
Agglutination of RBC
IgM
Paroxysmal Cold Hemoglobinuria in child
IgG
Attached at 4 degrees & activates at 37degrees
Altered color of urine at cold temp.
Biphasic antibidy = Donath Landsteiner Ab
Intravascular hemolysis
Aplastic anaemia caused by drugs
Chlorpromazine
Allopurinol
Diclofenac
