Benign Hematology
Learning Objectives
Author: Steven Naymagon
Editor: Lauren Peccoralo
I.
General Learning
Objectives
A.
Clinical Skills - Patient Care
1.
Take a thorough history and physical in a patient with anemia,
thrombocytopenia
and/or leukopenia.
2.
Order the appropriate laboratory tests in the appropriate order to make a
diagnosis in
the case of anemia, thrombocytopenia and/or leukopenia.
3.
Evaluate and work-up patients with coagulopathies (bleeding disorders)
and
hypercoagulable
states (i.e. DVTs, PEs)
B.
Interpersonal Skills
1.
Counsel patients and families with chronic hematologic diseases such as
sickle cell
disease and ITP
2.
Describe the risks and benefits of different types of transfusions to
patients
3. Work with team and consultants in
a constructive and polite manner to
work-up and manage hematologic issues.
C.
Procedural Skills
1.
Order and read a peripheral blood smear
2. Obtain appropriate consent and
order a blood transfusion for a patient
*Intern Focused Topics
II.
General Medical
Knowledge
1.
Understand the basic work-up of anemia and be able to interpret key
laboratory parameters including, but not limited to, the following*:
a)
Key findings on a peripheral smear
b)
MCV, RDW, reticulocyte count, reticulocyte index
c)
Fe, TIBC, Ferritin, Fe/TIBC ratio, MCV/RBC ratio
d)
B12, Folate, MMA, Homocysteine
e)
Bilirubin, LDH, haptoglobin, Coombs test
f)
Hemoglobin Electrophoresis, Immunofixation
g)
Reading:
(1) Bain BJ. Diagnosis from the Blood
Smear, NEJM, 2005*
http://eresources.library.mssm.edu:2368/cgi/content/full/353/5/498
2.
Sickle Cell Disease*:
a)
Understand the genetic and molecular mechanisms of
SS Dz and the
causes of vaso-occlusion.
b)
Understand role of fetal hemoglobin in severity of
disease and target
of therapy.
c)
Be able to recognize and anticipate the
complications of SS Dz
including vaso-occlusive pain crisis, acute chest
syndrome,
infection, etc.
d)
Know how to manage acute exacerbations and chronic
complications
of SS Dz
e)
Readings:
(1) Steinberg, MH. Management of
Sickle Cell Disease, NEJM, 1999*
(2) Frenette and Atweh. Sickle cell
disease: old discoveries, new concepts, and future promise, J of Clin Invest,
2007*
http://eresources.library.mssm.edu:2152/articlerender.fcgi?tool=pubmed&pubmedid=17404610
B. Non-Malignant
White Blood Cell Disorders
1.
Be familiar with the differential diagnosis of leukocytosis (infection,
malignancy, inflammatory states, drugs, collagen vascular disease, etc.)*
2.
Know how to interpret a WBC differential (left-shift, lymphocytosis,
eosinophilia, etc.) and how to proceed with the work-up of a particular
WBC line predominance*
3.
Know the causes of neutropenia (infection, drugs, autoimmune, etc.) and
the
indications for using neupogen and the evidence for its use.
4.
Readings:
a)
Abramson and Melton. Leukocytosis: Basics of
Clinical Assessment. AFP. 2000*
b)
Smith, TJ, et al. 2006 Update of Recommendations for
the Use of White Blood Cell Growth Factors: An Evidence-Based Clinical Practice
Guideline. J Clin Oncol. 2006
http://jco.ascopubs.org/cgi/content/full/24/19/3187
C. Platelet
Disorders
1.
Compare and contrast the clinical presentation (history, PE findings,
etc.) of
bleeding caused by a platelet disorder and bleeding caused by a
coagulation factor defect*
2.
Be familiar with commonly encountered quantitative platelet disorders*:
a)
ITP: making the diagnosis and therapeutic options
b)
HIT/T: know when to consider it, the molecular
mechanism,
management
c)
Other: hypersplenism, drugs, collagen vascular dz,
malignancy,
infection, etc.
3.
Understand the spectrum of HUS → TTP*:
a)
Recognize the clinical and laboratory findings that
suggest the
diagnosis
b)
Understand the molecular mechanism of the disorder
– ADAMTS13
c)
Know what should be done (and not done) for these
patients
4.
Be familiar with the commonly encountered qualitative platelet
disorders*:
a)
Recognize the acquired causes of platelet
dysfunction (medications,
uremia, liver disease, etc.)
b)
Understand the role of von WillebrandÕs factor in
the coagulation
process and the rationale of treatment with
desmopressin
5.
Readings:
a)
Cines, DB and Bussel, JB. How I treat idiopathic thrombocytopenic
purpura (ITP). Blood, 2005*
b)
Arepally, GM and Ortel, TL. Heparin-Induced
Thrombocytopenia. NEJM, 2006*
c)
George, JN. Thrombotic Thrombocytopenic Purpura.
NEJM. 2006*
http://eresources.library.mssm.edu:2368/cgi/content/full/354/18/1927
d)
Sadler, JE. Von Willebrand factor, ADAMTS13, and
thrombotic thrombocytopenic purpura. Blood. 2008
e)
Mannucci, PM. Treatment of von WillebrandÕs Disease.
NEJM. 2004
http://eresources.library.mssm.edu:2368/cgi/content/full/351/7/683
D. Coagulopathies
1.
Recognize the acquired and inherited coagulopathies*:
a)
Acquired: Liver Dz, drugs, Vit K deficiency, factor
inhibitors
b)
Inherited: Hemophelia, vWF deficiency, factor inhibitors
c)
Understand the indications for using FFP, vitamin K
(PO, SQ, IV),
factor replacement
d)
Describe the mixing study and its utility
2.
DIC*:
a)
Recognize the clinical and laboratory manifestations
b)
Understand the pathophysiology of DIC
c)
Distinguish between acute and chronic DIC
d)
How is DIC treated? What is the role of FFP,
cryoprecipitate,
platelets, APC, etc.
3.
Readings:
a)
Levi, M. Disseminated Intravascular Coagulation.
NEJM. 1999*
b)
Toh, CH and Dennis, M. Disseminated intravascular
coagulation: old disease, new hope. BMJ. 2003.
http://eresources.library.mssm.edu:2789/cgi/content/full/327/7421/974?view=long&pmid=14576251
E.
Hypercoagulable States
1.
Recognize the acquired risk factors for hypercoagulability (malignancy,
immobilization, medications, pregnancy, APS, vasculopathies, etc.)*
2.
Understand the pathophysiology, clinical manifestations and treatment of
APS*
3.
Recognize the most common inherited hypercoagulable states (Factor V
Leiden, Prothrombin mutations, AT III deficiency, Protein C&S
deficiency, etc.)*
4.
Describe when you should be suspicious of a thrombophilia and the timing
of
testing.*
5.
Know the indications for anticoagulation, the drug of choice, the
duration of
treatment, and INR goals for various prothrombotic states*
6.
Readings:
a)
DahlbŠck, B. Advances in understanding pathogenic
mechanisms of thrombophilic disorders. Blood. 2008*
b)
Levine, JS. The Antiphospholipid Syndrome. NEJM.
2002*
http://eresources.library.mssm.edu:2368/cgi/content/full/346/10/752
F. Transfusion
Medicine
1.
RBCs:*
a)
Know the indications for pRBC transfusion and the
transfusion goals
for patients with CAD? In the ICU? With CKD?
b)
Understand when to administer irradiated or
leukoreduced blood
products
2.
Platelets:*
a)
Know the indications and contra-indications for
platelet transfusion
and the goals for the average patient, in the
setting of infected,
when bleeding, pre-procedure
b)
Describe how to choose between pooled and single
donor platelets.
3.
Factors:*
a)
Know the indications for giving FFP and
cryoprecipitate
4.
Transfusion complications:*
a)
Know the most commonly transmitted infections and
what is the risk
b)
Know how to pre-treat patients prior to transfusion.
c)
Recognize the indications for stopping a transfusion
and how to
manage transfusion reactions
d)
Understand the pathophysiology, clinical
manifestations, and treatment of TRALI.
5.
Readings:
a)
Regan F, Blood Transfusion Medicine. BMJ. 2002*
http://eresources.library.mssm.edu:2789/cgi/content/full/325/7356/143?view=long&pmid=12130612