27.6.2013(pathology) 50 Flashcards
Mode of inheritance of CGD
70% X linked
30% AR
Most potent stimulator of naive T cell
Mature dendritic cells
Autoimmune disorders common in CGD
ITP
JRA
discoid lupus is common in x linked CGD
Late complications of CGD
Nodular regenerative hyperplasia
Portal hypertension
Major diagnostic criteria for multiple myeloma
Plasmacytoma on tissue biopsy
Bone marrow plasmocytosis greater than 30%
M protein spike
IgG>3.5g/dL
IgA>2g/dL
Urine light chains greater than 1g per 24 hours
Minor diagnostic criteria for multiple myeloma
Bone marrow plasmocytosis btw 10-30% Lytic bone lesions Low levels of M protein spike Lower than normal levels of immunoglobulins IgM less than 50mg/dL IgA less than 100mg/dL IgG less than 600mg/dL
International staging system of multiple myeloma
Stage 1(beta 2 micro globulin less than 3.5mg/L and serum albumin greater than or equal to 3.5g/dl) Stage 3(beta 2 micro globulin more than or equal to 5.5mg/L) Stage 2 is between 1 and 3
Diff btw MM and waldenstrom macroglobulinemia
No lytic bone lesions No hypercalcemia Hepatomegaly,splenomegaly,lymphadenopathy Hyper viscosity syndrome M spike is IgM
Plasma cell leukemia
Plasma cells more than 20% in peripheral blood
Asymptomatic or smouldering myeloma
No end organ involvement
M protein more than 3g/dL
Bone marrow plasmocytosis more than 10%
MGUS
No CRAB
Plasmocytosis less than 10%
M protein spike less than 3g/dl
Non secretory myeloma
Absence of M spike in serum or urine
Bone marrow plasmocytosis more than 10%
CRAB+
Symptomatic myeloma
Hypercalcemia Renal failure Anemia Lytic changes in bones Bone marrow plasmocytosis more than 10% M spike more than 3g/dl
Indolent MM
All features of multiple myeloma except end organ damage
Rx of multiple myeloma,transplant candidates
-VAD
vincristine,adriamycin,Dexamethasone
-High dose pulsed glucocorticoids
-thalidomide,Dexamethasone
Rx of multiple myeloma in non transplant candidates
Cyclophosphamide/melphalen+prednisolone
Novel therapies for MM
Bortezomib
Lenalidomide
INF A
BRCA chr location
BRCA 1(chr 17q) BRCA 2(chr 13q)
BRCA mutation
BRCA1 breast and ovary Ca in women,prostate Ca in men
BRCA 2 male breast cancer
Gum infiltration is common in which AML
M5
Chloroma is seen in
M2 AML
Most frequent cytogenetic change in MDS in Adults
5q deletion
Most frequent cytogenetic change in MDS in children
Monosomy 7
Favourable cytogenetics in AML
t(8;21) M2
Inv 16 M4
t(15;17) M3
M4 AML is also known as
Naegli type
Cells in M3 AML
Faggot cells
Markers of granulocytic sarcoma
Chloroma CD117 CD43 Lysozyme MPO
Most common type of AML
M2