Haematology Flashcards
1
Q
Haemopoiesis sites
A
3rd week of intrauterine life- yolk sac
3rd month of intrauterine life- liver
4th month of intrauterine life- bone marrow and liver
Just before birth- bone marrow
2
Q
CD marker to identify hematopoietic stem cell
A
CD-34
3
Q
Myeloid series
A
- Myeloblast
- Promyelocyte: largest cell
- Myelocyte
- Metamyelocyte
- Band form
- Neutrophil
Size 🔽 from promyelocyte to neutrophil
4
Q
Leucopenia
Agranulocytosis
A
🔽 in WBC county Usually due to neutropenia Cause: 1. Aplastic anemia 2. Drug toxicity Agranulocytosis: clinically significant 🔽 in WBC count
5
Q
Leucocytosis
A
🔼 WBC count
Reactive proliferation of WBC
6
Q
Neutrophilia
A
🔼 neutrophil count Normal neutrophil count: 40-70% Cause: 1. Acute infections 2. Tissue injury like MI, burns 3. Bacterial infections 4. Myeloproliferative disorders
7
Q
Eosinophilia
A
Normal count: 1-6% Causes: 1. Type 1 hypersensitivity like allergy 2. Parasitic/ helminthic infections 3. Tropical pulmonary eosinophilia
8
Q
Basophilia
A
Normal: 1% Causes: 1. CML 2. Lymphocytosis • TB • viral infections • chronic infections 3. Lymphoproliferative disorders like CLL, ALL
9
Q
Monocytosis
A
Normal: 2-8% Causes: 1. Malaria 2. Endocarditis 3. Rickettsiosis 4. Viral infections
10
Q
Peripheral smear in sepsis or infection
A
- Toxic granule:
Course & dark granule - Dohle bodies:
Patches of dilated endoplasmic reticulum
11
Q
Neutrophil classification based on lobes
A
1. Hypersegmented: >5 lobes seen In megaloblastic anemia due to vitamin B12 deficiency 2. Hyposegmented: <2 lobes seen Pseudo pelger huet cell In myelodysplastic syndrome
12
Q
Morphological abnormalities in May Hegglin anomaly
A
- Giant platelets
- Low platelet count
- Inclusion within the cytoplasm
13
Q
Abnormalities with granules in neutrophil
A
Chediak Higashi syndrome:
Dark purplish granule in giant granules in neutrophils
Alder Reilly abnormalities:
Most of the neutrophils have granules that obscure the nucleus
14
Q
Classification of WBC neoplasms
A
1. Lymphoid: ALL Chronic lympho proliferative disorders 2. Myeloid: AML Myeloproliferative disorders Myelodysplastic syndrome MDS/MPN 3. Dendritic cells: Langerhan’s histiocytes
15
Q
Acute lymphoblastic leukemia
A
WHO definition:
>20% blasts in bone marrow and peripheral blood
Based on markers
FAB definition:
>30% blasts in bone marrow and peripheral blood
Based on morphology of blasts
16
Q
Lymphoblast (🆚 myeloblast)
A
Smaller Scanty cytoplasm (🆚 moderate) No granules Auer rods absent Course, clumped chromatin: most prominent Inconspicuous nucleoli Stain: PAS +ve
17
Q
Myeloblast (🆚 lymphoblast)
A
Larger size Moderate cytoplasm Granules present Auer rods: hallmark Open up/homogenous chromatin: most prominent 2-5 prominent nucleoli Stain: MPO, Susan black B, NSE +ve
18
Q
Acute lymphoblastic leukemia
ALL
A
M/C cancer in children Age: 2-9 years Clinically: 1. Anemia ➡️ pallor, fatigue 2. 🔽 no of WBC - infections 3. 🔽 platelets - bleeding tendency 4. Hepatosplenomegaly due to extramedullary hematopoiesis 5. Bone pain (eternal tenderness) 6. CNS involvement 7. Testicular involvement 8. Lymph node involvement
19
Q
FAB classification of ALL
A
1. L1: Small homogenous blasts Scanty cytoplasm Inconspicuous nucleoli 2. L2: Large, heterogenous Moderate cytoplasm Prominent nucleoli 3. L3: Large Burkitt’s ‘lymphoma-like’ blasts Vacuoles in blue 🔵 cytoplasm
20
Q
Differences b/w ALL types (FAB classification) based on their occurrence and prognosis
A
L1: M/C Best prognosis 🍀ly L3: Least common Worst prognosis
21
Q
WHO classification of ALL
A
1. B-ALL: ☄️ M/C with better prognosis 🍀ly Usually children No mediastinal invasion Loss of function mutation In PAX-5, E2A or EBF 🧬 2. T-ALL: Adolescents Mediastinal invasion can be present Gain of function mutation in NOTCH I 🧬 Poor prognosis
22
Q
ALL
Lab diagnosis
A
1. CBC Hb and PLC 🔽 TLC 🔽 or 🔼 2. Peripheral smear: >20% lymphoblasts 3. Bone marrow aspiration: >20% lymphoblasts In aleukemic leukemia: No blasts in peripheral smear but on bone marrow aspiration >20% blasts
23
Q
Stains for ALL
A
- PAS +ve called DOT/BLOCK positivity
In AML-M6 shows diffuse PAS +ve - T-ALL are acid phosphatase positive
24
Q
Markers of ALL
A
- Of both pre B and pre T lymphocytes: TdT+ Terminal deoxynucleotidyl transferase
- B-ALL: CD19, 10, PAX-5
- T-ALL: CD1, 2, 5, 7
- CD-10 called CALLA Common ALL Ag
25
ALL
| treatment
1. Stem cell transplantation
2. Chemotherapy: VAPD
Vincristine
L Asparaginase
Prednisolone
Doxorubicin
3. Intrathecal methotrexate for CNS prophylaxis
26
ALL prognostic factors
| Good prognostic factors (🆚 bad = everything else)
1. Age: 2-9 years
2. Females
3. Whites
4. L1 subtypes
5. B-ALL
6. CNS, testis, spleen not involved
7. Hyperdiploidy (>50 chr) 🆚 hypodiploidy
8. Trisomy 4,7,10, t(12:21) 🆚 t(9:22)
9. TLC < 1,00,000/ml
27
AML
| age and clinical presentation
```
Age: 15-45 years
Clinically:
1. Non specific:
• Pallor, fatigue, 🔼 infections, bleeding tendencies, hepatosplenomegaly
• Usually no CNS, testes or lymph node involvement
2. Specific:
• Gun hyperplasia/bleeding
• DIC
• Chloroma or granulocytic sarcoma
```
28
AML
| risk factors
1. Chemicals and radiation ☢️
2. Genetic syndromes:
• Fanconi’s anemia
• Bloom’s syndrome
• Down’s syndrome
29
AML
| FAB classification
```
AML M0 AML undifferentiated
M1 AML w/o maturation
M2 AML with maturation
M3 acute promyelocytic leukemia
M4 acute myelomonocytic leukemia
M5 acute monocytic leukemia
M6 acute erythro leukemia
M7 acute megakaryoblastic leukemia
```
30
Special features of some types of AML
```
AML M0 is MPO -ve
AML M2:
• Pathogenesis t(8:21)
• M/C FAB type of AML
• Maximally associated with chloroma
AML M3:
Best prognosis
```
31
Myeloblastoma
Chloroma
Granulocytic sarcoma
```
Soft tissue involvement of AML
Comprised of myeloblasts and granulocytes
Green due to MPO +ve
M/C site: 👁 ➡️ proptosis
Arbiskov cells:
Presence of monocytes in a chloroma
```
32
AML M3
| Acute promyelocytic leukemia
```
Associated with t(15:17):
PML gene and RARA-α gene ➡️
AML RARA fusion protein ➡️
🔽 vitamin A level ➡️
🔽 conversion of promyelocytes-myelocyte ➡️
🔼 promyelocytes
```
33
Characteristics of cells of promyelocytes
* Have plenty of cytoplasmic granules and are usually hypergranular (azurophilic granules)
* A hypogranular variant also exists
* Few granules combine - Auer rods
* Criss cross pattern of Auer rods: Faggot cells
* Auer rods and faggot cells
34
AML M3
complication
treatment
Complications:
Granules contain thrombotic material ➡️ breakage of granules causes its release ➡️ DIC
Treatment:
All trans retinoic acid ATRA + arsenic trioxide
35
AML M4,5
```
Stains:
• MPO +ve
• NSE +ve, a stain for monoblasts
Strongly associated with gum bleeding
AML M5 is M/C AML in infants 👶
Associated with skin involvement:
Leukemia cutis
```
36
AML M6
Also called Di Gugleimo disease
| Diffuse PAS +ve
37
AML M7
```
Secrete PDFG ➡️ myelofibrosis ➡️ dry tap on bone marrow aspirate
Associated with Down’s syndrome
CD 41+, CD 61+
Budding cytoplasmic margins can be seen
Least common type of AML
```
38
WHO classification of AML
```
1. AML with recurrent genetic abnormalities:
• t(8:21), t(25:17)
• inv 16 or t(16:16)
• t(11q:V)
• with normal cytogenetics and mutated NPM
2. AML therapy related: 🔽 prognosis
3. AML with MDS like features
4. AML not otherwise specified:
all except AML M3
```
39
WHO classification of AML with recurrent genetic abnormalities
1. With t(8:21) RUN XI / ETO fusion gene: AML M2
2. With t(15:17) PML / RARA fusion gene: AML M3
3. AML with inv 6 out t(16:16) CBFB / MYHII fusion gene:
AML M4 (eosinophilia)
4. AML with t(11q:V) MLL fusion gene
5. AML with normal cytogenetics and mutated NPM
40
AML
| lab diagnosis
1. CBC:
🔽 Hb, TLC, platelets
2. Peripheral smear and bone marrow aspirate:
• >20% myeloblasts
• <20% with t(8:21), t(15:17) or inv. 16
3. Stains
4. Markers CD 13, 33, 117+, MPO +
41
AML
| stains
1. MPO+ myeloperoxidase
2. SBB+ Sudan black B
3. NSE+ monoblasts
AML M6 is diffuse PAS+
42
AML
| treatment
1. Definitive Rx: stem cell transplantation
| 2. For AML M3 ATRA +Arsenic trioxide
43
Biphenotypic leukemia
If blasts show both myeloid lineage markers as well as lymphoid markers, it is called biphenotypic
• Myeloid lineage markers: CD 13, 33, 117+, MPO+
• Lymphoid lineage markers: CD 19, 20, 10+, PAX 5+, CD 2, 3, 5, 7+
