Path 2

Question 1

Del11/13/17 & tri12, CD5, hypo gamma globulin, more mature, smudge cells; prolymphocytes & paraimmuniblasts; >5E9. Can be DLBCL vs EBV, woodwork, organic chemicals; GOF BRAF, upreg ANX1/lipocortin; splenomeg, DRY TAP; tx w/ chemo or BRAF inhib.

Answer 1

CLL (also know SLL and mono B cytosis) vs HCL

Question 2

Triple neg, CALR on 19p13, MPL activating TPO-R -> upreg JAK2 on 9p24; del20, tri8. Thrombosis & hem; inc PLT/megakaryocytes; tx w/ ASA or cytoreduction

Answer 2

ET

Question 3

Leukoerythroblastosis, EMH confirmed by bx, a/v thrombosis, TERMINAL OSTEOSCLEROSIS; I/III collagen on silver reticulin; nucleated RBC & teardrops, dry tap; ruxol vs chronic/accel/blast crisis, Philly, TdT+, pseudoGaucher, imatinib

Answer 3

PMF vs CML

Question 4

Germinal center, small cleaved centrocytes, big noncleaved centroblasts, LAD wax/wane. T14;18 & 2;18 -> BCL2, t3;14 -> BCL6, inactiv MLL2. Tx chemo

Answer 4

FL

Question 5

Del5/7/8, RUNX alpha, SPLICING FACTORS, RAS/JAK2; <20% blasts, dyserythroid/gran/mega; tx supportive & HCT

Answer 5

MDS

Question 6

Genes for trisomic MM/MGUS vs igH MM/MGUS vs MGUS/SM. Mature vs immature vs Dutch vs Russell/Mott. CD markers?

Answer 6

Gain odd number chrm vs 11;14 for CCND1, 6;14 for CCND3, 4;14 for FGFR3 vs RAS, del17, MYC. Clock face/spoke wheel vs big nucleus, dispersed chromatin vs intranuclear inclusion vs cyto grapelike inclusion/cluster Russell. CD38/138, +56 if neoplastic

Question 7

Describe CRAB for MM

Answer 7

HyperCa -> secrete RANKL & MIP1a; renal insufficiency -> light chain cast nephropathy, tubular casts w/ TammHorsafall; anemia; bone lytic lesions, osteolysis -> CT/MRI/PET > XR/skel survey

Question 8

How does MGUS become MM? 2 types of MGUS?

Answer 8

IL6 & IGF1 -> MAPK/P13K/JAK STAT. Only igM aka Waldenstrom vs igGADE or only light chains

Question 9

POEMS syndrome

Answer 9

Periph neuropathy, organomeg, endo, monoclonal protein in plasma, skin/hyperpig. Osteosclerotic/lytic lesions -> pathological fx. Bc of sural n & bone (CT/MRI guided) -> diffused light chain restricted plasma cells

Question 10

Febrile, neuro; CD25, IL2,; CD8 & Mac’s phag erythroid & gran > thrombo precursors; high AST/ALT & TG. Tx w/ immunosupression & mild chemo

Answer 10

HLH

Question 11

No extra cutaneous, 3 stages -> CCR4/10, skin bc shows cerebriform nuclei, Pautrier abscess vs into blood, epidermotropism, skin & LN bx show cerebriform nuclei; 3ad of pruritus, LAD, malig T cells

Answer 11

Mycosis fungoides vs Sezary syndrome

Question 12

vs epidermotropism, Pautrier-LIKE; bone lytic lesions, hyperCa, purpuric skin lesions, oncogenic retrovirus vs GOF STAT3, pale baso/azurophilic granules infilt bone marrow/liver/spleen -> pancytopenia

Answer 12

ATLL/HTLV1 vs LGL (know T cell vs NK cell CD markers)

Question 13

Cells found in thymic cortex vs medulla

Answer 13

Thymocytes, epith cells vs thymic myloid cells, dendritic cells, Hassall corpuscles

Question 14

AR UNC13D, PRF1, STXBP2/11; bone marrow bc/smear show erythroid hyperplasia, phag of eryhtroid & gran > thrombotic precursors -> inc ferritin, AST/ALT, TG, bili, CD25, neuro probs. Tx w/ immunosuppression & mild chemo

Answer 14

HLH