WBC Disorders Flashcards
Stain of
1) Myeloblast
2) Lymphoblast
1) MPO, Auer rods - AML
2) Terminal deoxynucleoitidal transferase - ALL
Blast cells in
- normal
- leukaemia
1-2%
> 20%
Risk factors of leukaemia
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 drugsACUTE LYMPHOBLASTIC LEUKAEMIA (ALL)
MC in child Sudden onset of symptoms : Pancytopenia / fatigue / fever Hepatosplenomegaly Lymphadenopathy STERNAL TENDERNESS*****
Genetic defect in ALL
Mutations : Hyperploidy Hypoploidy Trisomy 4/7/10 } good prognosis t (12:21);
(4 : 11) ;
(9 : 22) - poor prognosis
Diagnosis of ALL
BM exam : IOC
hypercellular
Blasts >20%
Lymphoblasts - TdT +ve, PAS +ve
Blood exam : TLC inc. Hb dec. Platelets dec. LYMPHOBLAST +/-
Immunophenotyping
Lymphoblasts not detected in peripheral circulation
Aleukemic leukemia
Immunophenotyping
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
Prognosis of ALL
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
Rx of ALL
Anti cancer drugs V - Vincristine A - Asparaginase P - Prednisolone D - Daunorubicin
Methotrexate—> intrathecal route
Allogenic bone marrow transplantation
Germinal centre gives 3 NHL
Follicular lymphoma
Diffuse large B - cell lymphoma
Burkitts lymphoma
Mantle zone lymphoma
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
Marginal zone lymphoma
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
Follicular lymphoma
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
Diffuse large B cell lymphoma
Most common
BCL-6 inc.
H/O Follicular lymphoma
dec. immunity (HIV/ transplant)—> EBV
HHV-8 (AIDS)—-> primary effusion lymphoma
Burkitts lymphoma
Max risk of mitosis
t (8;14)—-> C-MYC overactivity
t (2;8)
t (8;22)
Tumor lysis syndrome
LN biopsy : starry sky appearance **
Types of Burkitt lymphoma
Endemic (EBV)
Sporadic
HIV associated
Worst prognosis
Hairy cell leukaemia
B cell tumor
BM ; spleen ; liver
M»_space; F
Massive splenomegaly
Risk of infection (Atypical mycobacterium)
Pancytopenia
Hairy projections on phase contrast microscopy
Red pulp of spleen involved
Hepatosplenic lymphoma
Hairy cell leukaemia
Hairy cell leukaemia stain
TRAP + ve
Immunophenotyping in hairy cell leukaemia
ANNEXIN A1
CD 11 +
CD 25 +
CD 103 +
Bone marrow exam in hairy cell leukaemia
Aspiration : DRY TAP
Biopsy : Honey comb appearance
Fried egg appearance
Cutaneous T cell lymphoma
CD 4 T cell
Blood : Sezary syndrome
Skin : Epidermotropism = Pautrieres micro abscess ==== Mycosis fungoides
Appearance of tumor cell in cutaneous T cell lymphoma
Cerebriform nuclei
Why is skin involved in cutaneous T cell lymphoma?
Term most imp in this type of lymphoma
CLA + (cutaneous leukocyte antigen)
CCR 5 / CCR 10
Generalised exfoliative erythroderma
Anaplastic large cell lymphoma
Child
Young adult
HALL MARK CELLS = horse shaped nuclei
ALK gene (Chr.2 p)
CD 30 +
Good prognosis
Langerhans cell histiocytosis
3 subtypes
BRAF mutation
L cells : Birbeck granules = tennis racket appearance
Markers : CD 1a / S-100 / HLA-DR
Letterer- Siwe disease
< 2 yrs
Mutifocal multisystem
Lytic lesions (bone)
Skin lesions
Seborrheic dermatitis
Eosinophilic granuloma
Unisystem
Handschuller - Christian Triad :
Exopthalmos
Calvarial defects
DI
Pulmonary Langerhan cell Histiocytosis
Adult smokers
Handschuller - Christian Triad seen in
Eosinophilic granuloma
Genes mutated in CLL
11q 13q** 17p - deletion
12q - trisomy
NOTCH gene gain of function
Somatic hyper mutation
ZAP-70
Lymphoma not associated with radiation exposure
Chronic lymphocytic leukaemia
B cells in CLL secrete cytokines which cause structural defect in cytoskeleton
TNF alpha
TGF beta
Decrease normal activity of BM
VIMENTIN defect —-> inc. fragility
Concentration of antibodies in CLL
Hypogammaglobulinemia
Infections
Auto antibodies against self antigen
CLL causes which type of anemia
AIHA
CLL effect on platelets
Autoimmune thrombocytopenia
Blood investigation in CLL
Anemia
Platelets dec.
TLC inc.
Absolute lymphocyte count >5000 cells / micro litre
Test in CLL for RBC attached with antibodies
Coombs test
Smudge cells on peripheral smear and Convent girl appearance seen in
Also called as
CLL
Basket cells
Parachute cells
BM exam in CLL
Myeloid cells dec.
Erythroid cells dec.
Lymphoid precursors inc.
LN biopsy in CLL
Effacemmt / damage of LN bcoz of infiltration of tumor cells
Focal accumulation == PROLIFERATION CENTRES==== pseudo follicles
IOC in CLL
Flow cytometry
CD 19/20/21/23
CD5 +
Distinguish CLL from Mantle cell lymphoma by
CD5+ and CD23- in Mantle cell lymphoma
CD5+ and CD23+ in CLL
Additional mutation in CLL
Richter syndrome—–> DLBCL
LN size inc.
Spleen size inc.
Good & worst prognosis in CLL
13q & 17p
Rx in CLL
Fludarabin
Rituximab
Ibrutinib
Chronic lymphocytic leukaemia
CLL
Naive B cell
Post germinal centre B cell
Small lymphocytic leukaemia SLL
Peripheral B cell
MC in adults
