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Pathology > WBC Disorders > Flashcards

WBC Disorders Flashcards

1
Q

Stain of
1) Myeloblast

2) Lymphoblast

A

1) MPO, Auer rods - AML

2) Terminal deoxynucleoitidal transferase - ALL

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2
Q

Blast cells in

  • normal
  • leukaemia
A

1-2%

> 20%

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3
Q

Risk factors of leukaemia

A 
Genetic : 
-Fanconi anemia 
-Bloom syndrome 
-Ataxia telengiectasia
-Down syndrome -------->ALL (overALL)
                                              AML<3 yrs 
Acquired : 
Radiation exposure 
Chemicals : benzene, smoking
Infections : EBV, HTLV-1 
Drugs : Anti cancer drugs
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4
Q

ACUTE LYMPHOBLASTIC LEUKAEMIA (ALL)

A 
MC in child 
Sudden onset of symptoms : 
Pancytopenia / fatigue /  fever 
Hepatosplenomegaly 
Lymphadenopathy 
STERNAL TENDERNESS*****
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5
Q

Genetic defect in ALL

A 
Mutations : 
Hyperploidy 
Hypoploidy
Trisomy 4/7/10 } good prognosis 
t (12:21);

(4 : 11) ;

(9 : 22) - poor prognosis

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6
Q

Diagnosis of ALL

A

BM exam : IOC
hypercellular
Blasts >20%
Lymphoblasts - TdT +ve, PAS +ve

Blood exam : 
TLC inc.
Hb dec.
Platelets dec. 
LYMPHOBLAST +/-

Immunophenotyping

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7
Q

Lymphoblasts not detected in peripheral circulation

A

Aleukemic leukemia

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8
Q

Immunophenotyping

A 
Pre-B ALL : good prognosis 
More common, 
BM involved , 
3 yrs, 
EBF/ PAX-5 
CD 19/20
Pre-T ALL : poor prognosis 
Less common, 
Thymus, 
puberty, 
NOTCH gene mutation 
CD 1/2/5/7
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9
Q

Prognosis of ALL

A 
Good : 
White 
1-10 yrs 
Girls 
Less blasts 
PRE-B ALL
Bad : 
Black 
< 1 yr / > 10 yrs 
Boys 
More blasts 
Thymus / Testis 
PRE-T ALL
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10
Q

Rx of ALL

A 
Anti cancer drugs 
V - Vincristine 
A - Asparaginase
P - Prednisolone
D - Daunorubicin

Methotrexate—> intrathecal route

Allogenic bone marrow transplantation

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11
Q

Germinal centre gives 3 NHL

A

Follicular lymphoma
Diffuse large B - cell lymphoma
Burkitts lymphoma

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12
Q

Mantle zone lymphoma

A

Naive B cell
t (11;14) —> cyclin D1 overactivity causing inc in cells

Diffuse lymphadenopathy

CD 5 +
CD 23 -
Cyclin D1 +ve

If cyclin D1 -ve = SOX-11

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13
Q

Marginal zone lymphoma

A 
MALT - GIT / Lungs  
EXTRANODAL - Orbit 
Indolent 
Chronic B cell stimulation -
H.pylori ; 
 Autoimmune : RA / Sjogren syndrome / Hashimotos thyr.

t (11;18)

INITIAL : antibiotics

MALT : Ileum (Payers patches)
MALTOMA : Stomach

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14
Q

Follicular lymphoma

A

t (14;18)—–> BCL-2 inc.
MLL-2 mutation

Inc. cells
Painless lymphadenopathy
Indolent

CD 15/20 + ve
BCL-2 +
CD 5 - ve

Centroblasts ; Centrocytes ; Buttock cells

FL——-> DLBCL

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15
Q

Diffuse large B cell lymphoma

A

Most common
BCL-6 inc.
H/O Follicular lymphoma

dec. immunity (HIV/ transplant)—> EBV
HHV-8 (AIDS)—-> primary effusion lymphoma

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16
Q

Burkitts lymphoma

A

Max risk of mitosis
t (8;14)—-> C-MYC overactivity
t (2;8)
t (8;22)

Tumor lysis syndrome

LN biopsy : starry sky appearance **

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17
Q

Types of Burkitt lymphoma

A

Endemic (EBV)
Sporadic
HIV associated

Worst prognosis

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18
Q

Hairy cell leukaemia

A

B cell tumor
BM ; spleen ; liver
M&raquo_space; F

Massive splenomegaly
Risk of infection (Atypical mycobacterium)

Pancytopenia
Hairy projections on phase contrast microscopy

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19
Q

Red pulp of spleen involved

A

Hepatosplenic lymphoma

Hairy cell leukaemia

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20
Q

Hairy cell leukaemia stain

A

TRAP + ve

21
Q

Immunophenotyping in hairy cell leukaemia

A

ANNEXIN A1

CD 11 +
CD 25 +
CD 103 +

22
Q

Bone marrow exam in hairy cell leukaemia

A

Aspiration : DRY TAP

Biopsy : Honey comb appearance
Fried egg appearance

23
Q

Cutaneous T cell lymphoma

A

CD 4 T cell

Blood : Sezary syndrome

Skin : Epidermotropism = Pautrieres micro abscess ==== Mycosis fungoides

24
Q

Appearance of tumor cell in cutaneous T cell lymphoma

A

Cerebriform nuclei

25
Q

Why is skin involved in cutaneous T cell lymphoma?

Term most imp in this type of lymphoma

A

CLA + (cutaneous leukocyte antigen)

CCR 5 / CCR 10

Generalised exfoliative erythroderma

26
Q

Anaplastic large cell lymphoma

A

Child
Young adult

HALL MARK CELLS = horse shaped nuclei

ALK gene (Chr.2 p)

CD 30 +

Good prognosis

27
Q

Langerhans cell histiocytosis

3 subtypes

A

BRAF mutation

L cells : Birbeck granules = tennis racket appearance

Markers : CD 1a / S-100 / HLA-DR

28
Q

Letterer- Siwe disease

A

< 2 yrs
Mutifocal multisystem
Lytic lesions (bone)
Skin lesions

Seborrheic dermatitis

29
Q

Eosinophilic granuloma

A

Unisystem

Handschuller - Christian Triad :
Exopthalmos
Calvarial defects
DI

30
Q

Pulmonary Langerhan cell Histiocytosis

A

Adult smokers

31
Q

Handschuller - Christian Triad seen in

A

Eosinophilic granuloma

33
Q

Genes mutated in CLL

A

11q 13q** 17p - deletion

12q - trisomy

NOTCH gene gain of function

Somatic hyper mutation

ZAP-70

34
Q

Lymphoma not associated with radiation exposure

A

Chronic lymphocytic leukaemia

35
Q

B cells in CLL secrete cytokines which cause structural defect in cytoskeleton

A

TNF alpha
TGF beta

Decrease normal activity of BM

VIMENTIN defect —-> inc. fragility

36
Q

Concentration of antibodies in CLL

A

Hypogammaglobulinemia

Infections
Auto antibodies against self antigen

37
Q

CLL causes which type of anemia

A

AIHA

38
Q

CLL effect on platelets

A

Autoimmune thrombocytopenia

39
Q

Blood investigation in CLL

A

Anemia
Platelets dec.

TLC inc.

Absolute lymphocyte count >5000 cells / micro litre

40
Q

Test in CLL for RBC attached with antibodies

A

Coombs test

41
Q

Smudge cells on peripheral smear and Convent girl appearance seen in

Also called as

A

CLL

Basket cells
Parachute cells

42
Q

BM exam in CLL

A

Myeloid cells dec.
Erythroid cells dec.

Lymphoid precursors inc.

43
Q

LN biopsy in CLL

A

Effacemmt / damage of LN bcoz of infiltration of tumor cells

Focal accumulation == PROLIFERATION CENTRES==== pseudo follicles

44
Q

IOC in CLL

A

Flow cytometry

CD 19/20/21/23
CD5 +

45
Q

Distinguish CLL from Mantle cell lymphoma by

A

CD5+ and CD23- in Mantle cell lymphoma

CD5+ and CD23+ in CLL

46
Q

Additional mutation in CLL

A

Richter syndrome—–> DLBCL

LN size inc.
Spleen size inc.

47
Q

Good & worst prognosis in CLL

A

13q & 17p

48
Q

Rx in CLL

A

Fludarabin
Rituximab
Ibrutinib

51
Q

Chronic lymphocytic leukaemia

CLL

A

Naive B cell
Post germinal centre B cell

Small lymphocytic leukaemia SLL

Peripheral B cell
MC in adults

Pathology (2 decks)

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