Hematology - RBC Flashcards
1
Q
Hematopoiesis requires
A
Hematopoietic stem cells(HSC)
2
Q
Hematopoietic stem cells
A
Identified by CD34 marker
Pleuripotent stem cells
Have property of self renewal
3
Q
Hematopoiesis in fetal life
A
Starts from 3rd week
Takes place in Yolk sac
4
Q
Hematopoiesis after 3 months
A
Usually takes place in Liver and Spleen
5
Q
Hematopoiesis after birth
A
Bones (Bone marrow)
6
Q
Hematopoiesis after birth
A
Bone marrow
7
Q
HSC leads to formation of
A
Lymphoblast and
Myeloblast
8
Q
Lymphoblast
A
Lymphoid progenitor cells
15micron in size
Condensed nucleus (No nucleoli)
Cytoplasm without granules
Gives birth TO LYMPHOCYTES
9
Q
Myeloblast
A
Myeloid progenitor cells
20micron in size
Nucleus not condensed (prominent nucleoli)
Cytoplasm with granules
Gives birth to RBC, WBC (except Lymphocytes), Platelets
10
Q
Lymphoblast size
A
15 micron
11
Q
Myeloblast size
A
20 micron
12
Q
Prominent nucleoli is seen in
A
Myeloblast
13
Q
Lymphoblasts gives birth to
A
Lymphocytes
14
Q
Myeloblast gives birth to
A
RBC
WBC Except Lymphocytes
Platelets
15
Q
Most important hormone for RBC production
A
Erythropoietin
16
Q
Factor needed for formation of granulocytes
A
Granulocyte colony stimulating factor (g-CSF)
17
Q
Important cytokine for Platelets formation
A
IL-11
18
Q
Bone marrow Examination includes
A
BM Aspiration
BM Biopsy
19
Q
BM Aspiration Shows
A
Cell morphology
Enumeration
20
Q
BM Biopsy shows
A
Cellularity
Fibrosis
Checks infiltrative disorders
21
Q
Sites of Bone marrow Examination in Adults
A
M.c - Posterior superior iliac spine
- Also in sternum at level of 2nd IC (Only BM Aspiration)
22
Q
Site of Bone marrow Examination in obese people’s
A
Anterior superior iliac spine
23
Q
Site of BM Examination in Child (<2yrs)
A
Tibia
24
Q
Anticoagulant used for BM Examination
A
EDTA (doesn’t affects cell morphology)
25
Defeciency of RBC, WBC And platelets together
Pancytopenia
26
Stages of RBC Development
Myeloid stem cells - Colony forming unit-Erythroid (CFU-E) - Erythroblast - Normoblast(Early, intermediate and late) - Reticulocyte - RBC
27
Most erythropoietin sensitive cell
CFU-E
28
Hemoglobin appeared first in which cell with electron microscopy
Erythroblast
29
Hemoglobin 1st detection by routine staining
Intermediate Normoblast
30
Nucleus is absent in
Reticulocytes and RBCs
31
1st non-nucleated cell in RBC Development
Reticulocyte
32
Last nucleated cell in RBC Development
Late Normoblast
33
Normoblast types
Early (Basophilic) Normoblast
Intermediate (Polychromatophilic) Normoblast
Late (Orthochromatic) Normoblast
34
Which hormone decreases Hepcidin levels and increases Iron absorption
Erythroferrone
35
Reticulocytes are stained by
Supravital staining - New methylene blue
36
Normal amount of Reticulocytes
1-2% of RBC
37
Reticulocytes time for maturation
1 day
38
Absolute Reticulocyte count
= % Reticulocytes/100 x RBC count
39
Corrected Reticulocyte count
Reticulocytes (%) x Hb(patient) / Hb(Normal)
40
Reticulocyte production index (RPI)
Corrected Reticulocyte count / maturation factor or time correction
41
RPI < 2.5 Shows
Decreased proliferation or Maturation disorders
42
RPI < 2.5 Shows
Decreased proliferation or Maturation disorders
43
RPI > 2.5 (Increased reticulocytes) shows
Hemolytic anemia
44
RBC Normal shape and size
Biconcave shape and 7-8micron in size
45
Most hemoglobin present in RBC
Periphery
46
Parameters of RBC
MCH (Mean cell Hb)
MCV (Mean cell volume)
MCHC
RDW (Red cell distribution width)
47
MCH indicates
Avg Hb in RBC
Normal - 27-33pg
48
MCV Normal value
80-100fl
49
Microcytosis
MCV <80fl
50
Macrocytosis
MCV >100fl
51
MCV is
= Hematocrit x 10 / RBC
52
MCHC =
MCH/MCV
53
In case of anemia MCHC decreases except
Hereditary spherocytosis (increases)
54
Abnormal shape of RBC known as
Poikilocytosis
55
Variations in RBC shape and size
Anisocytosis
56
Index of Anisocytosis
RDW
57
Normal RDW
11.5-14.5
58
Anemia can be classified on the basis of
Decreased production of RBC
Blood loss
Hemolytic anemias (premature destruction)
59
Anemia classification on the basis of Size
Microcytosis (<80fl)
Macrocytosis (>100fl)
Normocytic (80-100fl)
60
Microcytosis anemia includes
Sideroblastic anemia
Iron deficiency anemia
Thalassemia
Anemia of chronic diseases (in late stages)
61
Macrocytosis anemia includes
Liver disease
Hypothyroidism
Myelodysplasia
Cell Maturation disorder
B12 and Folic acid deficiency
Alcohol
62
Normocytic anemia includes
Kidney failure
Anemia of chronic diseases (in early stages)
Myelofibrosis
Metastasis
63
Most common cause of anemia
Iron deficiency anemia
64
Iron absorption takes place in
Duodenum
65
Iron ferric form (Fe3+) converted into Ferrous form (Fe2+) by which enzyme
Cytochrome B reductase
66
Transmembrane protein that transports iron from inside of a cell to outside of cell
Ferroportin
67
Blood plasma glycoprotein that play central role in iron metabolism and responsible for ferric ion delivery
Transferrin
68
Iron stored in the body in the form of
Ferritin and Hemosiderin
69
Hemosiderin is stained by special stain named
Prussian blue stain - Perls reaction
70
Normal serum iron levels
100-200mg/dl
71
Total iron binding capacity
300-360mg/dl
72
Serum ferritin is inversely proportional to
Serum transferrin
73
Causes of IDA
Decreased intake of iron (M.C)
Increased requirement
Blood loss
74
Stage 1 of IDA
Decrease of iron in storage site - Serum ferritin level decreases (Earliest indicator)
75
Stage 2 of IDA
Changes in iron profile (No change in shape of RBC)
Serum iron decreases, % transferrin saturation decreases but Total iron binding capacity (TIBC) increases
76
Stage 3 of IDA
Iron deficiency anemia
Morphological changes
Size of RBC decreases, pale in color
77
Clinical features of IDA
Palpitations, weakness
Decreased work capacity
Koilonychia (soft nails)
PICA (Abnormal eating habits)
78
Plummer vinson Syndrome
Triad of IDA, Esophagus web and Glossitis
79
Gold standard method of diagnosis of IDA
BM Examination
80
Blood sample results in IDA
MCV Decreases
MCh Decreases
MCHC Decreases
Deceased osmotic fragility - in severe IDA
Serum ferritin decreases
Serum iron decreases
% Transferrin saturation decreases
TIBC increases
81
IDA is which type of anemia
Microcytic Hypochromic anemia
82
RBC Protoporphyrin levels in IDA
Increases because due to low iron levels Protoporphyrin will not be able to form Heme So there will be increased level of Free protoporphyrin in RBC
83
Mentzer index =
MCV / RBC Count
84
Mentzer index in IDA
>13
85
Mentzer index in Thalessemia trait
<13
86
Treatment of IDA
Treat primary cause
Iron supplementation
87
Serum transferrin receptor ratio / Log(ferritin) in IDA
>1.5
88
Serum transferrin receptor ratio / Log(ferritin) in Anemia of chronic diseases
<1.5
89
Risk factors of Anemia of chronic diseases (AOCD)
Chronic infections (TB)
Chronic inflammations (RA)
Cancers/Malignancy
All these leads to release of Cytokines
90
Cytokines responsible for AOCD
IL-6 (Most common) , IL-1 And TNF-Alpha
91
Effect of Cytokines on Bone marrow in AOCD
Decreases erythroid precursors(low erythropoietin) and that leads to decreases RBC production - Anemia
92
Type of anemia due to effects of Cytokines on Bone marrow
Normocytic Normochromic Anemia (No. Of RBC decreases but no change in morphology)
93
Effect of Cytokines on Liver in AOCD
Increases ferritin, Hepcidin levels and Decrease in transferrin levels
94
Increased Hepcidin levels
Inhibits Ferroportin which inhibits iron utilisation in RBC
95
Type of anemia due to effect of cytokines on Liver
Microcytic Hypochromic Anemia
96
Iron profile in AOCD
Serum ferritin increases
Serum iron decreases
% transferrin saturation decreases
TIBC decreases
97
Treatment of AOCD
Treat primary cause
Cancer patients - Erythropoietin
98
Anemia due to defect in heme metabolism
Sideroblastic anemia
99
Rate limiting enzyme in Heme metabolism
ALA Synthase
Needs cofactor Vit. B6
100
Congenital cause of Sideroblastic anemia
Decrease in enzyme activity
101
Acquired causes of Sideroblastic anemia
Alcohol intake
B6 deficiency
Lead poisoning
Decreased Copper levels
102
Sideroblastic anemia is type of which anemia
Microcytic Hypochromic anemia
103
Ringed sideroblast is seen in
Sideroblastic anemia
Myelodysplastic syndrome
Thalessemia
B12 deficiency
Some hemolytic anemias
104
Iron profile in Sideroblastic anemia
S. Ferritin increases
S. Iron increases
% transferrin saturation increases
TIBC Decreases
105
RBC morphological changes in Sideroblastic anemia
RBC decreases in size
Pale in color
106
BM Examination in Sideroblastic anemia shows
Ringed sideroblast
107
Treatment of Sideroblastic anemia
Treat primary cause
108
Hereditary spherocytosis mode of inheritance
Autosomal dominant inheritance
109
Normal RBC membrane proteins
Spectrin
Ankyrin
BAND 3
BAND 41
Glycophorin
110
Which RBC Membrane protein contribute most in shape
Spectrin
111
Which RBC Membrane protein is most abundant
Glycophorin
112
What happens with aging of RBC
RBC becomes less flexible and become more prone to splenic macrophages (Splenic phagocytosis)
113
Most common defect seen in which RBC membrane protein in Hereditary spherocytosis
Ankyrin
114
Most severe Hereditary spherocytosis seen in defect of which RBC Membrane protein
Spectrin
115
Decrease in life span of RBC in Hereditary spherocytosis
From 120 days to 10-15 days
116
Clinical features of Hereditary spherocytosis
Anemia
Splenomegaly
Jaundice
Positive family history
117
BM Examination in Hereditary spherocytosis
Shows increased activity of BM and increased level of Erythroid precursors
118
Blood test results in Hereditary spherocytosis
MCH Normal
Hb decreases
MCV Decreases
MCHC Increases
119
Peripheral smear of Hereditary spherocytosis
Central pallor absent
Spherical RBCs
120
Spherical RBCs can be seen in which conditions
Autoimmune hemolytic anemia
Hereditary spherocytosis
G6PD deficiency
Infections
121
Most common cause of Spherical RBCs
Autoimmune hemolytic anemia
122
Osmotic fragility in Hereditary spherocytosis
PINK test - INCREASES
123
Procedure of Autohemolyser
Take patient blood sample + 0.9% saline solution and wait for 48 hours
124
Result of Autohemolyser in case of Hereditary spherocytosis
>15% RBC Destruction
125
Result of Autohemolyser in case of Normal individuals
3-4% of RBC Destruction
126
Dye used for Flow Cytometry
5-EMA (Eosin maleimide) dye
127
Mean fluorescence in case of Hereditary spherocytosis
Decreases
128
Management of Hereditary spherocytosis
Removal of spleen - bczz main site of RBC Destruction
129
Increase in average life span of RBC after Splenectomy in Hereditary spherocytosis
Can increase upto 60days
130
Is there Change in shape of RBC after Splenectomy
No bczz hereditary
131
High level of Bilirubin due to chronic hemolysis can lead to
Pigment gall stones
Bilirubin binds with Calcium and increases risk of gall stone development
132
Most common metabolic disorder which leads to RBC Destruction
G6PD deficiency
133
G6PD Deficiency mode of inheritance
X linked recessive inheritance
134
G6PD normally helps in
Neutralizing Oxidative stress with the help of reduced glutathione
135
G6PD deficiency can leads to
Increased susceptibility to oxidative stress
136
G6PD deficient patients are sensitive to
Infections
Food - like Fava beans
Drugs - Primaquine, Sulfa drugs, Nitrofurantoin, Nalidixic acid
137
Heinz body
Indicative of oxidative injury to the erythrocytes
138
Stain used to see Heinz body
Supravital staining - Crystal violet
139
If denatured Hb in case of G6PD deficiency precipitates in RBC
Known as Bite cell or DEGMACYTE
140
If denatured Hb directly damages RBC
Intravascular hemolysis
141
If Denatured Hb forms abnormal connections with RBC
Decreased flexibility and destroyed by splenic phagocytosis
Extravascular hemolysis
142
Clinical features of G6PD deficiency
Weakness
Altered color of urine
143
G6PD variants
G6PD - B (Most common and stable)
G6PD - A+
G6PD - A-
G6PD M (least stable) - severe hemolysis
144
Blood investigation in G6PD deficiency shows
Low Hb levels
145
Peripheral smear in G6PD deficient patients on Supravital staining
Heinz body
146
Peripheral smear in G6PD deficient patients on Routine staining
Bite cell/DEGMACYTE
Blister cell
Spherocytes
147
G6PD estimation can be done by
ASSAY
Electrophoresis
Fluorescent spot test - sensitive and screening test
148
Benefits in G6PD deficient patients
Protection against Plasmodium falciperum(Malaria)
149
Management of G6PD deficiency
Treat primary cause if present
Observation
If taken drugs - discontinue
Self limiting condition
150
Hypoxemia due to increased RBC Destruction can trigger
Release of Erythropoietin from Kidney
151
Hemolytic anemia occurs
When There is imbalance between RBC Destruction and compensatory mechanism of bone marrow
152
Due to increased Bone marrow activity in Hemolytic anemia there is
Increased cellularity (No. Of cells increases)
Reticulocytes increases
153
Due to RBC Destruction in Hemolytic anemia we can see
Anemia
Increased serum LDH levels
Increased levels of unconjugated bilirubin - JAUNDICE
154
Destruction of iron in tubular cells of kidney known as
Renal hemosiderosis
155
Types of hemolytic anemias
Intravascular hemolytic anemia
Extravascular Hemolytic anemia
156
Site of RBC Destruction in intravascular hemolytic anemia
Systemic circulation
157
Site of RBC Destruction in extravascular Hemolytic anemia
Liver and spleen
158
Massive increase in size of liver and spleen seen in which hemolytic anemia
Extravascular Hemolytic anemia
159
Haptoglobin levels in Hemolytic anemia
Decreases in both intravascular (more) and extravascular Hemolytic anemia
160
Causes of Hemolytic anemia can be divided into
Intracorpuscular causes
Extracorpuscular causes
161
Examples of intravascular hemolytic anemias
Paroxysmal nocturnal Hburia(PNH)
G6PD Deficiency
162
Examples of Extravascular Hemolytic anemia
Sickle cell anemia
Thalessemia
163
Inherited intracorpuscular causes of HA includes
Hereditary spherocytosis
G6PD Deficiency
Sickle cell anemia
Thalessemia
164
Acquired intracorpuscular causes of HA includes
Paroxysmal nocturnal Hburia (PNH)
165
Immune mediated Extracorpuscular causes of HA includes
ABO RH incompatibility
Autoimmune HA
166
Non immune mediated Extracorpuscular causes of HA includes
Clostridium toxin
Snake venom toxin
Mechanical damage
167
Hemoglobinopathies includes
Sickle cell anemia
Thalessemia
168
Sickle cell anemia mode of inheritance
Autosomal recessive
169
In sickle cell anemia Glutamic acid is replaced by
Valine
170
Which chain of Hb is affected in sickle cell anemia
Beta chain (Point mutation)
171
Beta|Beta
HbA - Normal
172
Beta | Beta s
HbA ; HbS - Sickle cell trait
173
Beta s | Beta s
HbS - Sickle cell anemia
174
HbS in sickle cell anemia
Decreased RBC flexibility - Splenic phagocytosis
And is Sticky which can leads to vascular occlusion and can cause ischemic damage
175
Sickle cell anemia is which type of HA
Extravascular Hemolytic anemia
176
Effects of sickle cell anemia on bones
Dactylitis (Bone foot syndrome)
Avascular necrosis (femur)
177
Clinical features of sickle cell anemia
Skin - non healing ulcer
Retarded growth in childrens
Lungs - acute chest syndrome
178
Spleen in sickle cell anemia
Splenomegaly (Abdominal fullness)
Can leads to Autosplenectomy
179
Bodies seen in sickle cell anemia due to Calcium deposition and congestion in spleen
Ganma-gandy bodies
180
Osmotic fragility in SCA
Decreases
181
Peripheral smear in SCA
Drepanocytes (Sickle like cells)
182
HPLC full form
High performance Liquid chromatography
183
Thalessemia is which type of disorder
Quantitative
184
Alpha-Thalessemia occurs due to
Gene deletion - less frequent
185
Beta- Thalessemia occurs due to
Gene mutation - 80-90% more common
186
Beta gene isoforms
Beta - full synthesis of chains
Beta ° - No synthesis
Beta + - Partial synthesis of Beta chains
187
Most common mutation in Beta Thalessemia
Non sense mutation - immediate stop codon comes and stops synthesis
188
Mutations seen in Beta Thalessemia
Splicing mutations - MC cause of Beta + Thalessemia
Promoter mutations - makes fixed quantity of chains
189
Beta | Beta
Normal
190
Beta + | Beta
Beta ° | Beta
Thalessemia minor or trait
Asymptomatic, mild anemia
191
Beta + | Beta +
Thalessemia intermedia
Symptoms seen, moderate anemia
Often Requirement of blood transfusion
192
Beta ° | Beta °
Beta ° | Beta +
Beta + | Beta +
Thalessemia Major or Cooley's anemia
193
Signs of Thalessemia major
Hb <7g/dl
Symptoms +++
Severe anemia
Repeated history of blood transfusion
194
Pathophysiology of Beta Thalessemia
Excess alpha chains forms tetramers which precipitates in Normoblast and damages their membrane Leads to Ineffective erythropoiesis or if it( alpha tetramers) goes to systemic circulation can lead to Reticuloendothelial destruction
195
Thalessemia is which type of HA
Extravascular Hemolytic anemia
196
Effect of Thalessemia on bones
Increased BM compensatory activity - Extra medullary hematopoiesis - Chimpanji like facial appearance
197
Clinical features in Thalessemia major
Weakness
Splenomegaly
Hepatomegaly
Pallor
Dyspnea
Fatigue
198
Repeated blood transfusion in Thalessemia patient can leads to
Iron overload - secondary hemochromatosis
199
Blood results in Thalassemia major
Decreased Hb
MCH, MCV and MCHC Decreases
200
Peripheral smear in Thalessemia
Aniso-poikilocytosis
Target cells (Codocytes)
Basophilic stippling
Hovell jolly bodies
201
Peripheral smear in Thalessemia
Aniso-poikilocytosis
Target cells (Codocytes)
Basophilic stippling
Hovell jolly bodies
202
Osmotic fragility in Thalassemia
Decreases
203
Genetic methods used in case of Thalassemia
HPLC
Electrophoresis
Globin gene sequencing(molecular testing)
204
Best investigation or definitive diagnosis in case of Thalassemia majo4
Molecular testing - not available everywhere
205
Radiology signs in Thalessemia
Skull - Crew cut appearance or hair on end appearance
206
Treatment of Thalessemia major
Blood transfusion for whole life
Iron chelators to prevent iron overload
Allogenic bone marrow transplantation
207
Thalessemia intermedia
Less severe than Thalassemia major
Often history of blood transfusion
Hb - 7-10g/dl
208
Thalessemia trait
Mild anemia
Asymptomatic
No history of blood transfusion
209
Screening test for Thalessemia
Nestrof test - Checks osmotic fragility
210
Nestrof test in case of Thalessemia
RBC doesn't burst due to Decreased osmotic fragility - BLACK LINE NOT VISIBLE
211
Nestrof test in case of Normal
Normal RBC burst so BLACK LINE VISIBLE
212
Mentzer index in Thalessemia trait
<13
213
Hb HPLC in Thalessemia trait
Decreased HbA and Increased HbA2 (>3.5 g/dl)
214
Alpha Thalessemia
Relatively uncommon than Beta thalassemia
Gene deletion
215
Alpha,Alpha | Alpha,Alpha
Normal
216
Apha,Alpha | Alpha, -
Silent carrier (75%)
217
Alpha, Alpha| - , -
Alpha, - | Alpha, -
Mild symptoms
In 50%
218
Alpha, - | - , -
Beta 4 tetramer - Hb H disease - High affinity to O2 leads to Hypoxia
Extravascular hemolysis
219
Golf ball appearance is seen in
Hb H disease
220
- , - | - , -
Gamma 4 tetramer - Barts hemoglobin
High affinity for O2(Hypoxia), risk of congestive failure
221
Source of vitamin B12
Animal food
222
Absorption of Vitamin B12 is done by
Stomach - intrinsic factor
Pancreas
Gut bacteria
Ileum
223
Vit. B12 in case of Stomach surgery or in Pernicious anemia
Decreased level of intrinsic factor
224
Vit. B12 absorption if pancreas not working properly
Decreased absorption
225
Increased gut bacteria effect on Vit B12
Can consumes B12 and will not be available for absorption
226
Vitamin B12 absorption in ileum pathologies (IBD, Lymphoma)
Decreased absorption
227
Vitamin B12 plays role in
BM / Blood cells
GIT
Nervous system
228
Homocysteine converted to Methionine with the help of
Vitamin B12 and Folic acid
Methionine - Important for DNA formation
229
Methylmalonyl-CoA converted into Succinyl CoA with the help of
Vitamin B12
230
Succinyl CoA is important for
Myelin formation
231
Increased homocysteine levels can leads to
Endothelial cell injury - promote atherosclerosis
232
In BM Vitamin B12 is important for
Conversion of precursor cells into mature cells
233
Ineffective erythropoiesis in case of Vitamin B12 deficiency is due to
Increased no. Of cells (Hypercellular)
Pancytopenia
Increased no. Of immature cells
234
WBCs in case of Vitamin B12 deficiency
Stab cells (immature neutrophils)
Hypersegmented neutrophils
235
Platelets in case of Vitamin B12 deficiency
Abnormal shape
Increased in size
236
Effect of Vit. B12 deficiency on Nervous system
PNS - Paresthesias
CNS - Subacute combiner degeneration
237
Effects on GIT in Vit B12 deficiency
Megaloblastoid changes in epithelium (Epithelial atrophy)
Beefy tongue
238
Clinical features of Vit B12 deficiency
Anemia, fatigue, weakness
Mild Jaundice
Neurological manifestation
Increased skin pigmentation due to increased activity of Tyrosinase - increased melanin secretion
239
Peripheral smear of RBC in B12 deficiency
Size increases
Howell jolly body
Cabot ring
Basophilic stippling
240
Peripheral smear of WBC In B12 deficiency
Hypersegmented neutrophils
241
Serum homocysteine and Methylmalonyl-CoA levls in B12 deficiency
Increases
242
Treatment of vitamin B12 deficiency
Vitamin B12 supplementation
Iron/Folic acid
243
Treatment of vitamin B12 deficiency
Vitamin B12 supplementation
Iron/Folic acid
244
Pernicious anemia is which type of disorder
Autoimmune disorder
245
Autoantibodies formed in Pernicious anemia usually effects
Gastric fundus of stomach - lead to atrophic gastritis and High risk of development of cancer
Decreased level of intrinsic factor - Decreases vitamin B12 absorption
246
Diagnosis of Pernicious anemia
Autoantibodies
Blood/BM exam
Gastric biopsy
Schilling test
247
Schilling test
Radioactive vit B12 is given - excretion of B12 from urine (>8%)
248
Folate deficiency is due to deficiency of
Folic acid
249
Folic acid is usually absorbed from
Jejunum
250
Folic acid is needed in
Conversion of homocysteine to Methionine along with vitamin B12
251
Etiology of Folate deficiency
Reduced intake of green leafy vegetables
Increased requirement (Pregnancy, lactation)
Malabsorption syndrome
Malignancy
Drugs - Alcohol, OCPs, Phenytoin, Methotrexate
252
Clinical features of Folate deficiency
Anemia
No neurological symptoms
253
Blood test results in folate deficiency
S. Homocysteine elevated
S Methylmalonyl-CoA Normal
Serum folate levels decreases
254
Figlu test is useful for identification of
Folic acid deficiency
255
Folate deficiency during pregnancy can leads to
Neural tube defects in newborn
256
Treatment of folate deficiency
Give folate + B12 together
257
Autoimmune hemolytic anemia (AIHA) is type of which anemia
Extracorpuscular HA
258
AIHA is divided into
Warm AIHA
Cold AIHA
259
In Warm AIHA, Antibodies attaches to RBC at what temperature
37°C
260
Antibodies responsible in Warm AIHA
IgG >>> IgA
261
In Warm AIHA, RBC Destruction usually takes place in
Spleen >>>> Liver
RBC membrane loose - Spherical in size (Spherocytes)
262
According to site of RBC Destruction, Warm AIHA is classified into
Extravascular Hemolytic anemia
263
Etiology of Warm AIHA
Idiopathic
SLE/RA
Cancers
Drugs - Penicillin/Cephalosporin
Quinidine
Alpha-methyldopa
264
Clinicals features of Warm AIHA
Anemia
Jaundice
Splenomegaly
265
Diagnosis of Warm AIHA
Increased unconjugated bilirubin
Increased LDH levels
266
Peripheral smear in AIHA
Spherocytes (no central pale area)
267
Blood test in Warm AIHA
Auto Antibodies test - COOMB'S test direct or indirect
268
In Cold AIHA, antibodies attaches to RBC at what temperature
<37°C
269
Antibodies responsible for Cold AIHA
IgM >>> IgG
270
Cold AIHA can be classified into
Cold Agglutinin disease
Cold hemolysin disease
271
Site of RBC Destruction in Cold Agglutinin disease
Kupffer cells (LIVER)
272
Antigen against IgM in Cold Agglutinin disease
'I' antigen
273
Etiology of Cold Agglutinin disease
Idiopathic
Malignancy
Mycoplasma infection
Infectious mononucleosis
274
Clinical features of Cold Agglutinin disease
Acrocyanosis (Cyanosis in peripheral areas)
Jaundice
Anemia
275
Diagnosis of Cold Agglutinin disease
Slide test - RBC Forms clumps at low temperature
Chilled slide test
276
Antibodies responsible for Cold Hemolysin disease
IgG
277
Antigen in Cold Hemolysin disease against IgG
'P' Antigen
278
Antibodies activates at what temperature
When exposed to cold temperature (like 4°C)
279
Antibodies shows action at what temperature in Cold Hemolysin disease
It activates complement system When temperature comes back to 37°C
280
Activation of complement system in Cold Hemolysin disease leads to
Formation of Membrane attack complex(MAC) - RBC Destruction - Intravascular hemolytic anemia
281
Clinical features of Cold Hemolysin disease
Altered urine color
Mostly affect pediatric population
Anemia
Jaundice
282
Example of Intracorpuscular Acquired genetic defect
Paroxysmal nocturnal Hburia
283
PIG-A Gene produces which molecule
GPI-P
284
Proteins attached to GPI-P are
CD59
CD55
C8 binding protein
285
Function of proteins attached to GPI-P
Protection against complement system
286
In PNH there is defect in
PIG-A gene - GPI-P doesn't works properly - also not 1,2,3 protein - increased chances of damage due to activation of complement system
287
In PNH, there is deficiency of which blood cells
Pancytopenia (RBC, WBC And platelets)
288
Effect on RBC in PNH
Increased destruction by complementary proteins - Intravascular hemolysis - Hburia
289
Effect on WBC in PNH
Defective WBC - Increased risk of infection
LAP Score decreases
290
Effect on platelets in PNH
Thrombocytopenia
Dysfunctional platelets can undergo aggregation and leads to clot formation - increased risk of Thrombosis
291
Clinical features of PNH
Pancytopenia
Altered urine color (most darkest in early morning)
Anemia
Jaundice
Increased risk of infection
292
HAM'S Test
Pt. Blood sample + acidified serum - Change in pH - activates Complement system - RBC destruction
Used as screening test
293
Most important test for diagnosis of PNH
Flow cytometry
294
Flow Cytometry in PNH shows
Absence or presence of CD molecules
295
Dye used for Flow Cytometry in PNH
FLAER (Fluorescent labeled Aerolysin)
296
Biphenotypic cells are seen in
PNH
297
In PNH patient, in which quadrants we can see scattered cells
In Quadrant 1 and 3
298
PNH can progress into
Myelodysplastic syndrome
Acute myeloid leukemia
Aplastic anemia
299
Treatment of PNH
Eculizumab - inhibits activity of C5 convertase ( Complement system)
Bone marrow or stem cell transplant
300
Defect in Hematopoietic stem cells leads to which type of anemia
Aplastic anemia
301
Abnormal stem cells have
Decreased replication rate
Decreased rate of differentiation
New antigens activates T cells which leads to activation of Cytokines IFN delta, TNF alpha
302
Bone marrow activity in aplastic anemia
Bone marrow Hypoplasia ( Decreased BM activity)
303
Activation of T cells in aplastic anemia can leads to
Activation of Cytokines such as IFN- delta, TNF-alpha
304
Aplastic anemia can progress to
Myelodysplastic syndrome
AML
PNH - if T cells activation against GPI-P antigen
305
Inherited causes of Aplastic anemia
Telomerase enzyme defect
Fanconi's anemia
306
Fanconi's anemia
Autosomal recessive
DNA repair genes defet
Decreased kidney or spleen size
Bone defects
307
Acquired causes of Aplastic anemia
Immune mediated aplastic anemia
Idiopathic (MC)
308
Chemicals related causes of Aplastic anemia
Dose related Aplastic anemia - Alkylating agents, antimetabolite drugs, chloramphenicol
Dose unrelated AA - Gold salts, Chloramphenicol
309
Physical agents that can cause aplastic anemia
Radiation exposure
Viruses - CMV, varicella zoster, Epstein Barr
310
Clinical features of Aplastic anemia
Pancytopenia
Anemia, weakness
Petechiae, purpura
Splenomegaly never seen in AA
311
Blood test results in Aplastic anemia
Pancytopenia
Reticulocytopenia
312
Bone marrow exam in Aplastic anemia
BM Aspiration - "DRY TAP"
BM Biopsy - decreases HSC - HYPOCELLULAR BM
313
Treatment of Aplastic anemia
Bone marrow transplantation
If no suitable donor - antithymoside globulin
Cyclosporine
314
Chemita classification of Aplastic anemia
Non severe AA
Severe AA
Very sever AA
315
Non severe AA
When BM cellularity <25%
316
Severe AA
When BM cellularity <25% + any 2 out of 3 - ANC <500/mm3, Platelets <20,000/mm3, Reticulocytes <60,000/mm3
317
Very severe AA
Same as Severe AA except ANC <200/mm3
318
Very severe AA
Same as Severe AA except ANC <200/mm3
