WBC Disorders Flashcards
(48 cards)
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
