Hematology Flashcards
Approach to MH: Myeloid vs. Lymphoid - which blood components are affected in each?
- Myeloid: RBCs, Platelets, monocyte, granulocytes
- Lymphoid: B and T cells
Name 4 Myeloid Disorders. Risk of all myeloid disorders?
Myeloid Disorders
- AML: too many immature myeloid cells
- MPN: too many mature cells (PV, ET, CML)
- CMML: features of both MPN/MDS
- MDS: not enough myeloid cells > BM failure
Risk
- All can evolve into AML
Name 2 Lymphoid Disorders
- ALL: too many immature cells
- LPD: too many mature cells (CLL, lymphoma, WM)
- Plasma Cell Dyscrasias: too many plasma cells (MGUS, smoldering myeloma, MM; primary amyloidosis)
Which 3 cell types make up granulocytes?
- Eosinophils
- Basophils
- Neutrophils
Acute Leukemia:
- Etiology
- Presentation S&S
- Diagnosis
- AML vs. ALL?
Etiology
- De novo
- Secondary cause: prior chemo, transformed MDS/MPN, congenital disorders (Fanconi anemia, Down), Benzene
Presentation S&S
- BM failure: cytopenias - anemia (fatigue), thrombocytopenia (bleed), neutropenia (infxn)
- Systemic sx (d/t blasts): leukostasis, DIC, TLS
- Organ Dysfxn: skin>rash, mucosa>gym hypertrophy, CNS>neuro sx
Diagnosis
- >=20% blasts in peripheral blood or BM
- WHO classification relies on genetic mutations
AML vs. ALL
- Based on BM biopsy, flow cytometry, cytogenetics, molecular
- Auer rods onyl in myeloid neoplasms > auer rods never normal
Acute Leukemia: If blasts + auer rods + DIC you should think?
Acute Promyelocytic Leukemia (APL)
- Subtype of AML
- T(15;17) PML-RARA
- Most curable AML type
- Early mortality from DIC (ICH, blood clots)
- Urgent Tx: ATRA (all-trans retinoic acid)
Labs: high K+, PO4, UA, low Ca2+
- Diagnosis?
- Complications?
- Management?
Tumor Lysis Syndrome (TLS)
- Rapid destruction of tumor cells
- Usually after starting cytotoxic treatment
Complications
- AKI (from hyperuricemia > urate nephropathy)
- Seizures
- Arrhythmia
Treatment
- IVF (target U/O 80-100ml/m2/h)
- Manage hyperK, correct lytes
- Allopurinol or Rasburicase (if AKI, ++ UA [>535], no response to allopurinol (not in G6PD def)
What is Leukostasis? How common? S&S? Treatment?
Leukostasis
Medical emergency: tisse hypoxia + hypercoag 2/2 rigid sticky blasts causing microvasc obstruction > MOD
Etiology: most common in acute leukemia
- AML>ALL>CML»CLL
- AML if WBC >50-100
- Higher WBC in CML/ALL (>300-400)
- Rare in CLL
S&S: lungs/CNS most commonly affected
- Lungs: SOB, hypoxia
- CNS: confusion, visual changes
- DIC: common complication of APL
Treatment:
- IVF
- Cytoreduction (eg., hydroxyurea, leukophereses, induction chemo)
- TLS prophylaxis (allopurinol, Rasburicase)
- Avoid transfusions
Management of DIC: Non-APL vs. APL
Non-APL
- Platelets: no bleed = plt<10; bleed = plt<50
- FFP (15ml/kg): if bleed + PT or PTT >1.5x ULN
- Fibrinogen concentrate (4g): if bleed + fibrinogen <1.5
APL
- ATRA
- Platelets: if plt <30-50
- FFP (15ml/kg): if bleed (no PT/PTT target unless bleed)
- Fibrinogen concentrate: if fibrinogen <1.5
Which leukemia has high risk for CNS involvement and therefore intrathecal chemo +/- rads is indicated?
ALL
What are 4 types of MPN that you should know + mutations?
Types:
- CML, ET, PV, PMF
Philadelphia +ve [BCR-ABL, t(9;22)]
- CML
JAK2+, Philadelphia -ve
- >95% PV
- ~50% ET/PMF (primary myelofibrosis)
Calreticulin +ve (CALR)
- 30% of ET/PMF
All MPN can transform into?
- Acute leukemia
- Myelofibrosis
CML:
- Diagnosis?
- Phases?
Diagnosis
- PCR + for BCR/ABL t;(9;22) “philadelphia” chromosome
- BMBx to confirm phase/prog (cytogenetics)
Phases
Chronic
- Blasts <10%
- High WBC (mostly neuts + precursors)
- Splenomeg, wt loss, fever, night sweats
- Tx: TKi
Accelerated
- Blasts 10-19%; basophils >=20%
- Additional clonal cytogenetic abn in Ph+ cells
- Anemia, infection, extreme splenomegaly
- Worsening counts despite tx
Blast
- Blasts >=20% = acute leukemia
- Tx: as per acute leukemia + TKi
Polycythemia Vera (PV)
- S&S
- Diagnosis
- Risk stratification
- Treatment
S&S
- CBC: high Hb +/- WBC +/- Plt
- Erythromelalgia, aquagenic pruritus
- Arterial/VTE complications
Diagnosis: 3 major OR 2 major + 1 minor
Major
- Hb >165/160 (M/F) or HCT >49/48 (M/F)
- BM: hypercellular for age, trilineage growth
- JAK2 V617F (or JAK2 exon 12) mutation
Minor
- low serum EPO
Polycythemia Vera (PV)
- S&S
- Diagnosis
- Risk stratification
- Treatment
Risk Stratification
- Low: <60 and no thrombosis hx
- High: >60 or thrombosis hx
Treatment
Everyone:
- ASA81
- Optimize CV risk fx
- Hct <45% with phlebotomy
High risk:
- Cytoreduction with Hydroxyurea (interferon if young or pregnant)
- 2nd line: Ruxolitinib (RESPONSE trial) - pt resistant or intolerant to Hydrea
- VTE Hx: AC
- Arterial thrombosis hx: consider ASA BID
