prognosis

Question 1

agranulocytosis

Answer 1

Overwhelming infections can cause death within hours to days.

Question 2

neutropenia

Answer 2

-Increased susceptibility to bacterial and fungal infections

Question 3

Hemophagocytic Lymphohistocytosis (HLH)

Answer 3

Treated: 50% survive
—May have significant sequelae (renal damage: adults, mental retardation: children).

Untreated: grim prognosis, < 2 months survival if familial

Question 4

ALL (acute lymphoblastic leukemia)

Answer 4

o Poor Prognosis if
“ Age < 2 (MLL translocation).
“ T(9:22) Philadelphia ch.
“ Presentation in adolescence or adulthood.
“ Peripheral blood blast count > 100,000 (high tumor burden likely).
“ Detection of residual disease after therapy.

o	Favorable Prognosis if:
"	Age between 2 and 10 yo.
"	Low white cell count.
"	Hyperdiploidy.** 90% have this
"	Chromosome 4, 7, 10 trisomy.
"	Presence of t(12:21).

• leading cause of childhood death d/t cancer.
• 95% of children reach complete remission.

adults: Only 35-45% are cured d/t different molecular pathogenesis and inability to tolerate the necessary chemo regimen effective in children

Question 5

Richter synd (from C/SLL (chronics,small lymphocytic leukemia))

Answer 5

under 1yr

Question 6

C/SLL (chronics,small lymphocytic leukemia)

Answer 6

Median survival 4-6 yrs, 10 yrs in pts with Decreased tumor burden at diagnosis.

Poor Prognosis if:

• 11q, 17p deletions.
• Lack of somatic hypermutation.
• ZAP70 (+): augments Ig receptor signaling.
• NOTCH1 mutations.
• Richter syndrome.

Question 7

burkitt lymphoma

Answer 7

• Responds well to chemotherapy.
• Children and young adults are usually cured.
• Older adults have more guarded outcome

Question 8

follicular lymphoma

Answer 8

• waxing and waning course
• 7-9 yrs regardless tx
• progress DLBCL or burkitts –> 1 yr

Question 9

DLBCL (diffuse large B cell lymphoma)

Answer 9

• rapidly fatal w/o tx

- Treated: 60-80% complete remission, 40-50% cured.

Question 10

multiple myeloma (plasma cell myeloma)

Answer 10

Survival 4-7 yrs

If untx bone lesions: 6-12 mos

Question 11

solitary myeloma (plasmocytoma)

Answer 11

Bone lesion in same locations as MM → multiple myeloma in 10-20 yrs even if it is resected.

Extraosseous lesions in lungs, oronasopharynx or nasal sinuses → cured by local resection. (not onto MM)

Question 12

smoldering myeloma

Answer 12

75% progress (unpredictably) to multiple myeloma in 15 yrs.

Question 13

-lymphoplasmacytic lymphoma

Answer 13

4 yrs

Question 14

mantle cell lymphoma (MCL)

Answer 14

poor: 304 yrs

organ dysfunction d/t lack of effective chemo and tumor infiltration.

Question 15

hairy cell leukemia

Answer 15

excellent

Question 16

Peripheral T-cell Lymphoma, Unspecified

Answer 16

cure rare; worse prognosis than aggressive B cells

Question 17

Anaplastic Large Cell Lymphoma

Answer 17

good prog: kid and alk

bad prod: adult and alk-

Question 18

Adult T-cell Leukemia/Lymphoma

Answer 18

• Rapidly progressive disease, fatal in months to 1 yr despite aggressive chemo.
• If only skin is involved (rare), disease has a more indolent course

Question 19

Mycosis Fungoides & Sezary Syndrome

Answer 19

8-9 yrs

Question 20

Extranodal NK/T-cell Lymphoma

Answer 20

highly aggressive; poor in adv dis

Question 21

Hodgkin Lymphoma: Nodular Sclerosis Subtype
Hodgkin Lymphoma: Mixed Cellularity Subtype
Hodgkin Lymphoma: Lymphocyte Rich Subtype
Hodgkin Lymphoma: Lymphocyte Depletion Subtype
Hodgkin Lymphoma: Lymphocyte predominant Subtype

Answer 21

• excellent: stage 1, 2
• adv stage, but good prog
• very good-excellent
• adv stage, bad prog
• excellent: stage 1, 2, but recur

Question 22

AML w genetic abbr
AML w MDS-like features
AML therapy related
AML, NOS
Overall:

Answer 22

• AML w genetic abbr: favorable
• AML w MDS-like features: poor
• AML therapy related: very poor
• AML, NOS: intermediate
• overall: 60% remission with chemo, 5 yrs: 70-85% relapse.

Question 23

Myelodysplastic syndromes (MDS)

Answer 23

• 9-29 survive

- Worse outcomes: higher blast counts, more severe cytopenias with multiple clonal chromo Abn

Question 24

t-MDS

Answer 24

4.8 mos, high progression to AML

Question 25

Chronic Myelogenous Leukemia (CML)

Answer 25

• worse if not have phl chromo, in blast crisis, accelerated phase
• w/o tx: 3 yrs

Question 26

Polycythemia Vera (PCV or PV)

Answer 26

untx: death w/in mos

Question 27

Essential Thrombocytosis (ET)

Answer 27

12-15 yr survival

Question 28

-Multifocal Multisystem Langerhans Cell Histiocytosis (letterer-siwe Disease)

Answer 28

Untreated = rapidly fatal

5 yr survival: 50%