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What Ab are you looking for in inflammatory joint diseases?
ANA - anti-nuclear Ab
What is gaucher's disease and how would you diagnose?
- -lysosomal storage disease
- - "gaucher's cells" in BM, macrophages with too many lipids
An example of why you would see lymphadenopathy in:
inguinal, axillary, R supra, L supra, ant cervical, post cervical
- inguinal: cervical cancer
- axillary: breast cancer
- R supra: malig of esophagus
- L supra: gastric ca
- ant cervical: infectious
- post cerv: malignant
What are B symptoms (literally B-cell Sx)
- night sweats
- weight loss
List some causes of generalized lymphadenopathy
- Infectious: HIV, TB, mono
- Inflammation: SLE
What is on the DDx for reactionary lymphocytosis?
- physiological stress
- hypersensitivity to drugs
What is on the DDx for clonal lymphocytosis?
- monoclonal B-cell lymphocytosis (new disease)
- chronic lymphocytic leukemia
- other B or T cell lymphomas
When does Hodgkin's lymphoma happen?
bimodal peaks: one at 20, the other at 65
What is the clinical triad for MM
- high M-protein
- osteolytic bone lesions
- ++ myeloma cells in BM
What are the big 3 to watch out for in heme malignancies
anemia, leukocytosis, thrombocytopenia
What are the CRAB criteria for MM?
- Ca++ elevated
- Renal problems
- Bone disease (lesions)
6 major complications of all MPDs (MPNs)
- clonal evolution (e.g. AML)
- B symptoms
- bone pain
Where do you see auer rods and granules?
Clinical presentation of acute leukemia
- -B symptoms
- -SHORT (weeks to a few months) duration of symptoms
- -Big 3 (anemia, leukopenia (especially neutropenia), thrombocytopenia)
- -sometimes tissue infiltration and leukostasis
When is acute leukemia an especially emergent condition?
When there is DIC or tumor lysis syndrome
incidence peaks of ALL and AML in kids
Describe the chemo therapy in ALL
- 1) early intensive chemo to induce remission
- 2) long term chemo to prevent resistance and relapse
List three important aspects of palliative care
- -all patients with a progressive incurable illness could benefit from PC
- -PC applies to all ages
- -PC constitutes active care guided by family's expectations
List some reactive and clonal causes of lymphocytosis
- -physiologic stress response
- -monoclonal B-cell lymphocytosis (<5)
- -Other B or T cell leukemias
In which hematological malignancy do you often test positive for Philadelphia chromosome?
Philadelphia *C*rea*ML* cheese
When diagnosing polycythemia vera what are two good tests?
- 1) ABG to rule out hypoxia
- 2) test for a JAK2 mutation
What hematological malignancy has Auer rods?
What is the most common childhood malignancy? Prognosis?
Good prognosis (85% remission with chemo)
How do you treat CLL?
No chemo until they are symptomatic, then chemo is used only in a palliative manner
If you see a patient come in with a VERY high WBC count (>500) with blasts, what should you be thinking?
Describe tumor lysis syndrome
During Tmt of hematological malignancy, rapic cellular death releases intracellular contents, causing a ton of electrolyte disturbances
What is the diagnostic test of choice for NHL and HL?
excisional LN biopsy!
Which leukemia would you expect to see in the following ages?
< 13 years (but can present at any age group)
< 13 years (but can present at any age group): ALL
- 40-60 years: CML
- 60+ years: CLL, AML
How do you treat indolent NHL? aggressive NHL?
- indolent: palliative chemo, symptomatic
- aggressive: aggresively with a curative approach
Describe the ages you most likely will see Hodgkin's lymphoma
bimodal age dist
3rd decade and around 60
What is the classic triad of diagnostic criteria for Multiple Myeloma
- >10% plasma cells in the bone marrow
- M protein in serum or urine (not enough to dx on its own)
- Bone lesions
ALL is associated with which syndrome?
We ALL go DOWN together
What is the definition of acute leukemia?
greater than 20% blasts in the BM at presentation
What type of anemia does MDS cause?
What mutation is common to PV, IMF, and ET? What is the incidence in each?
- PV: 95%
- IMF: 50%
- ET: 50%
What are 3 things you see in idiopathic myelofibrosis?
- -teardrop cells
- -dry BM aspirate
- -leukoerythroblastic blood film (RBC and granulocyte precursors)
treatment of ALL vs AML? ALL treatment
ALL tmt: induction->consolidation->maintenance->CNS prophylaxis
- ALL vs AML
- 1) no maintenance in AML
- 2) no CNS prophylaxis in ALL
how does HL present? (6 chars)
painless, non-tender, firm, rubbery, enlargement of superficial cervical LN's
What cells are used to pathognomonically differentiate NHL from HL?
Reed-Sternburg cells are present in HL
What is the goal of NHL treatment in indolent NHL? aggresive NHL? tmt for each?
- indolent NHL: palliative/watchful waiting, R-CVP
- aggressive NHL: curative, R-CHOP
What does tmt of HL depend on? NHL?
HL depends on staging, NHL depends on hisological subtype (indolent or aggressive)
What is the natural history of CLL? What is it?
malignancy of mature B-cells
indolent, but incurable with a slow progression
What is the triad that you are looking for in the serum and the bones in multiple myeloma
- serum: spike in serum protein (M-protein)
- Bone marrow: >10% plasma cells
- osteolytic bone lesions
How is MGUS different from MM? same? how many undergo malignant transformation per year?
both have spike in M protein
MGUS doesn't have CRAB (same with SMM), <10% plasma cells in marrow
1% undergo transformation per year
How much does one unit of pRBCs raise Hct by? Hb?
List the three categories of NHL-associated conditions with an example of each.