High Yield info for final Flashcards
In which disorder is an allogenic bone marrow transplant indicated
Aplastic anemia
If you have a prolonged PT/INR, what type of vitamin deficiency do you suspect? Which drug inhibits this vitamin?
Vitamin K deficiency
Warfarin inhibits vitamin K
What is the most concentrated factor in cryoprecipitate
Fibringoen
Your patient presents with fever, chills, hypotension, flank and back pain, and bleeding following a transfusion. Which disorder do you suspect?
Acute hemolytic reaction
Your patient has Reed Sternberg cells. What do you suspect is his diagnosis?
Hodgkin’s lymphoma
*Bimodal distribution, see it in young adult population and older adults
*Alcohol consumption leads to pain in lymph nodes and bones
*Uses ann arbour staging
*To confirm Hodgkin’s diagnosis, you NEED a tissue (LN) biopsy
You have heinz bodies and bite cells on a smear. What is the diagnosis?
G6PD
Your patient has abnormal RBCs under hypoxic conditions. Which disease is this characteristic with
Sickle Cell anemia
Your patient has hard, fixed, painless lymph nodes. What are these associated with?
Malignant lymph nodes
Your patient has mobile/rubbery lymph nodes. What are these associated with?
Lymphoma
Your patient has painful, inflamed, and soft lymph nodes. What is this associated with?
Infection- non-cancerous
What are the 3 B symptoms of Non-Hodgkin lymphoma?
Fever (3+ days), night sweats, unintentional weight loss (10%)
What test do you use to distinguish B12 and Folate anemia?
MMA
Which test measures the intrinsic and common coagulation pathway
aPTT
- *Use a mix-in test to measure prolonged PTT and distinguish origin/diagnosis
Which test measures the extrinsic coagulation pathway
PT
Which drug inhibits the intrensic pathway
Heparin
Measure using PTT
factors 12, 11, 9, 8 ( hemophila A is a factor 8 deficiency, remember)
What drug inhibits the extrensic pathway
Warfarin= vitamin K antagonist
measure using PT
Factor 2, 7, 9, 10 are vitamin K dependent factors
Extrensic pathway are factors Tissue factor (3), 7
What factors are part of the common pathway
10, 5, 2( thrombin), fibrinogen, fibrin clot (1a)
If your PTT is long, which pathway is dysfunctioning?
Intrensic and common are both measured by PTT
If your PT is long, which pathway is dysfunctiongin?
Extrensic
Which special test do you use to confirm that the patient has autoimmune hemolytic anemia (And you suspect it after a blood smear)?
Coomb’s test
What protein is made in the liver that binds to free Hgb in the blood?
Haptoglobin
If haptoglobin is low, suggests hemolytic anemia. If haptoglobin is normal, nearly rules out hemolytic anemia ( very sensitive test)
Which disorder is associated with the BCR-ABL1 fusion and the philadelphia chromosome?
ALL
*NOTE that the philadelphia chromosome is heavily implicated in CML in ALL cases, but in ALL in SOME cases. Just so its confusing!
If you suspect a patient has ALL, which test do you order as the gold standard?
Bone marrow biopsy– need tissue to confirm issue
Auer rods
AML- Acute myeloid leukemia
Risk factors include prior chemo or radiation, benzene exposure
Smudge cells
CLL- Lymphocytes with Crushed Little Lymphocytes for Smudge cells
Adults over the age of 60 are most common age group, and most adults over 60 have gone through a divorce and have SMUDGED their mascara
Translocation from 9 to 22 (Philadelphia chromosome 22)
CML!
Some types of ALL can have philadelphia chromosome, but that is a different gene fusion not translocation
Characteristics of Multiple Myeloma electrophoresis and blood smear
M spikes on electrophoresis
Bence Jones proteins
have Rouleaux formation
BJ (Bence Jones) likes M&Ms (Multiple Myeloma/ M spikes)
Characteristics/symptoms are CRAB
C-HyperCALcemia
R- Renal dysfunction
A-Anemia
B-Bone lesions
How do you differentiate Multiple Myeloma from MGUS
Use CRAB
C-HyperCALcemia
R- Renal dysfunction
A-Anemia
B-Bone lesions
Functionally mature lymphocytes with incompetent monoclonal B lymphocytes
CLL
Has Smudge cells too
Which cell line causes Non-Hodgkin’s lymphoma
B cells 85% of the time (CD20) and T otherwise
Which cell line causes Hodgkin’s lymphoma
B cell origin
Your patient complains of bone pain when he drinks alcohol. Which test do you order next?
- Peripheral smear, see reed-sternberg cells, large cells that are multilobed owl eye nuclei.
- Lymph node biopsy is needed to confirm diagnosis
Mature B cell malignancy that can develop from MGUS
Multiple myeloma
MGUS–> SMM–> Multiple Myeloma
Also have clonal proliferation of plasma cells and increased monoclonal protein in the body
If you have an increase in mature, dysfunctional, B lymphocytes in the body, what disorder is this characteristic of?
CLL
If you have clonal proliferation of plasma cells and increased monoclonal protein in the body, what disorder does this indicate?
Multiple Myeloma and MGUS, differentiate using CRAB
What is the most common clotting disorder
Factor 5 leiden
What is the most common bleeding disorder
vWF
If you see schistocytes on a blood smear, what disorder do you expect?
TTP- most dangerous and common.
DIC also has schistocytes, but you have a PTT increased also with DIC
Schistocytes are irregularly shaped fragments of RBCs because of mechanical distruction or oxidative stress
Pathophys of schistocytes
Schistocytes are irregularly shaped fragments of RBCs because of mechanical distruction or oxidative stress
Which test measures average RBC volume
MCV
Which disorder is the beta globin chain of hemoglobin affected in
Beta thalassemia
Also sickle cell
Factor that’s deficient in hemophilia A
8
Retic count is increased in what type of anemia
Hemolytic anemia
must have functioning bone marrow to increase retics
This test measures percent of immature RBCs in blood and indicates bone marrow activity
Reticulocyte count
What test do you do after you do a CBC
Reticulocyte count
Virus associated with malignant lymphoma
EBV
Transfusion of PRBCs is indicated when Hgb falls below which number in stable patients without active bleeding
<7
Which blood product gives you coagulation factors and other proteins in plasma
FFP
Which blood component gives you insoluble coagulation factors (Fibrinogen, factor 8, facotr 13, vWF)
Cryoprecipitate
- Precipitated out of FFP
Indication for platelets transfusion
Platelets <10-20k
OR actively bleeding, or pre-operatively
Indication for FFP
INR of >1.8, active DIC, intra-op bleeding
Indication to give cryoprecipitate
Low fibrinogen
Indication for G-CSF transfusion
Neutropenia
1 unit of PRBCs will raise what value by which amount
raise HEMOGLOBIN by 1 g/dL
raise Hematocrit by 3%
1 unit of platelets will raise platelet count by how much
40-50k
1 unit of cryoprecipitate will raise fibrinogen levels by how much
70-100 mg/dL in 70kg recipient ( you give 5-10 unitis usually)
Life span of platelets
7-10 days
How long to raise iron levels in body
2-6 weeks
How many years of stores does the body have of B12
5 years
Class of immunotherapy drugs that target PD-1 pathways
Checkpoint inhibitors
CART is what type of chemo drug
Immunotherapy drug
BITE (Bi-specific T cell engagers) are what type of drug
Immunotherapy drugs
CAR-T are what type of drug
Immunotherapy drugs
Oncolytic viruses are what type of drug
Immunotherapy drugs
Targetted therapies are targetting what on the cancer?
Target specific antigens or mutations (DRIVER mutations) in cancer DNA)
Anti- HER2 is what type of cancer treatment
Targeted therapy
Monoclonal antibodies are what type of cancer treatment
Targeted therapies
Which cells are most likely to be impacted by chemotherapy drugs
Rapidly dividing cells
- Hair cells
- GI tract
- Skin
- Reproductive system
What is a monoclonal antibody that targets CD20 and treats lymphomas
CD20 inhibitors
Rutiximab (vitamin R)
Anyone with B cell dysfunction gets this
Type of anemia caused by b12 or folate
Megaloblastic
First clotting factor activated in intrinsic pathway
12
Excessive fibrosis in bone marrow, may lead to extramedullary hematopoiesis
Primary myelofibrosis
Mutation is in JAK2
Medication that is the antidote for warfarin toxicity
Vitamin K
Clonal proliferation of megakaryocytes and mostly due to JAK2
Essential Thrombocytosis
Polycythemia vera has the risk of transformation to what disorder
Myelofibrosis or AML
Is Polycythemia vera acquired or genetic
Acquired Myeloproliforative neoplasm (myeloid condition where blood production is deregulated)
What is the clonal driver of MPNS
JAK2
EXCEPT secondary polycythemia
Primary polycythemia refers to what
Specifically to polycythemia vera
Secondary polycythemia refers to what
Arises from underlying conditions that stimulate the body to produce more RBCs (often due to lack of oxygen)
Conditions include High altitude, certain tumors, smoking
JAK2 is in all types of MPNs except which one?
Secondary polycythemia
What are the 4 myeloproliferative neoplasms
Polycythemia Vera
Essential thrombocytosis
Primary myelofibrosis
Chronic myeloid leukemia
Autoimmune condition that causes hemolysis via IgG antibodies and positive Coombs test
Warm autoimmune hemolytic anemia
Anticoagulant protein that inhibits factor 2a
antithrombin 3
Vitamin essential for carboxylation of 2, 7, 9, 10
Vitamin K
Immunoglobulin that is the biggest and most responsible for early immune responses
IgM
Acute vs chronic infections with immunoglobulin presence
IgM on early immune responses
IgG on chronic (EBV or hepatitis)
What is the name of This Left supraclavicular lymph node that may indicate abdominal malignancy or gastric cancer
Virchow’s node
EPO stimulating agents are commonly used to treat anemia in this chronic condition
CKD
Also Anemia of chronic disease, HIV, and MDS, RA
This myeloproliferative neoplasm is associated with a mutation in the JAK2 gene in 95% of cases and leads to overproduction of RBCs
Polycythemia Vera
Symptoms in Polycythemia vera vs Essential Thrombocytosis
Erythromelalgia is more commonly seen in PV than ET
Aquagenic puritis is ONLY in PV
PV has increased RBCs, Increased HcT, may show normal or slightly elevated WBC and platelet counts, ALWAYS has low EPO, Hypercellular bone marrow
ET has Increased platelets, Normal Hgb/Hct with Normocytic RBCs, NO erythrocytosis. JAK mutation
In Secondary polycythemia, which hormone is elevated in response to hypoxia
EPO
Hydroxyurea is used to increase fetal Hgb production in this condition
Sickle cell anemia
Person with this blood type can only receive blood from donors with same blood type due to both Anti a and anti b antibodies
O blood
Which blood is the universal donor
O-
Transfusion reaction with hypovolemia, pulmonary edema, hypertension, JVD, and cardiac or renal compromise
TACO
Essential thrombosis may experience this painful sensation in the hands of feet due to microvascular thrombosis
Erythromelalgia
Can occur in Essential thrombosis AND polycythemia vera, more common in PV
Chemotherapy drug that’s red devil that is a component of ABVD
What does it treat?
Doxyrubicin- ruby- red devil
Anthracycline is class, also called andramycin
Treats Hodgkin’s lymphoma
Transfusion reaction with acute respiratory distress, non cardiogenic pulmonary edema within 6 hours of transfusion
TRALI
Normocytic anemia, low iron, high ferritin, normal TIBC, increased inflammation
What are the inflammatory markers in this condition?
Anemia of chronic disease
CRP and IL-11 are the inflammatory markers
Symptom where you crave for non food items like ice
PICA
Seen in IDA
Clotting factor that is known as fibrin-stabilizing factor, crosslinks fibrin strands to stabilize a clot
Factor 13
Immunotherapy that modifies a T cell to target Cancer cells to treat lymphoma
CAR-T therapy
What is an inherited disorder leads to excessive iron absorption and deposition in tissues
Hereditary hemochromatosis
Paraneoplastic electrolyte disturbance associated with small cell lung cancer, can be triggered by chemotherapy agents like cyclophosphamide and vincristine
SIADH
Your patient has decreased urine output, neurological deficits, and increased weight without edema after chemotherapy and infection with pneumonia. What could they be suffering from?
SIADH
- Risk factors are chemothrapy (Vincristine and cyclophosphamide, ifosamide), infection with bacterial pneumonia/fungal disease, and cancer with CNS tumors.
Treatments= fluid restrictions and diuretics
What is a left shift
Increased number of bands, often indicative of bacterial infection or severe immune response
Emergency caused by metastatic tumors, back pain, motor weakness, incontinence
Spinal cord compression
Bone marrow disorder characterized by dacrocytes
primary myelofibrosis
Leukoerythroblastic smear- nucleated reds, blasts that scar out bone marrow to fill in cells that arent supposed to be there
Bone marrow disorder characterized by dacrocytes (teardrop cells), hematopoiesis, splenomegaly. What is it, and how do you treat it?
Treatment is JAKIFY
Primary myelofibrosis is JAK+
Bone marrow biopsy will show dry tap
Life threatening complication occurs spontaneously in patients with hemophilia, severe headache, vomiting, altered mental status, loss of consciousness
Intercranial hemmorhage
Patients with this inherited hemoglobinopathy are at risk for complications with splenic infarction, susceptibility to infections, etc
Sickle cell anemia
these patients also get dactylitis, parvovirus
This Emergency is characterized by a fever greater than 100.4, ANC is <500/uL, requires prompt antibiotic therapy to prevent sepsis
Febrile neutropenia
Prolongation of PT and PTT suggests defect in this path including factors 1, 2, 5, and 10
Common pathway
Patients with this myeloproliferative disorder are advised to avoid iron supplements, as increased iron may exacerbate excessive RBC production
Polycythemia vera
Treatment is phlebotomy
Fragile, damaged lymphocytes, smudge cells, are characteristic on PBSs in this type of leukemia
CLL- Crushed little lymphocytes- smudged
Auer rods are characteristic of this disorder
AML
Hyperlobed neutrophils is characteristic of this disorder
MDS
Megaloblastic anemia is what, and what disorders is this caracteristic of?
Megaloblastic- B12/Folate deficiency
very large RBCs
This process, where blood cell production occurs outside the bone marrow, is seen in thalassemias, myelofibrosis, and hemolytic anemias
Extramedullary hematopoiesis
Often triggered by E. coli, characterized by acute kidney injury, microangiopathic
HUS
Fusion gene, formed by by translocation between 9 and 22 is hallmark of CML
BCR-ABL fusion, philadelphia chromosome
Viral infection known for causing fever and joint pain, leads to transient pancytopenia due to bone marrow suppression
Parvovirus B19
causes transient pancytopenia, sickle cell crisis
Severe adverse effect of bleomycin and is limitation in its use in cancer treatment
Pulmonary toxicity
Term that refers to reduction in RBCs, WBCs, platelets, commonly seen in bone marrow failure
Pancytopenia
This condition results from autoantibody-mediated destruction of platelets
ITP
On a smear, you see thrombocytopenia, but everything else is normal
Taking oral iron supplements every other day instead of daily improves absorption because this hormone level increases when iron is abundant
Hepcidin
Oncologic emergency caused by large tumors leading to rapid cell lysis, hyperuricemia, hyperkalemia, hyperphosphatemia, and HYPOcalcemia
Tumor lysis syndrome
Inherited disorder causes fragile RBCs leading to splenomegaly
Hereditary spherocytosis
Compression of mediastinal mass can cause emergency, facial swelling
SVC syndrome
Normal or elevated level of protein rules out hemolysis as it is typically decreased in hemolytic anemia due to role in binding free hemoglobin
Haptoglobin
This type of anemia is caused by autoantibodies destroying cells that produce intrensic factor
pernicious anemia (B12 deficiency)
What disorder arises as a result of n ADAMTS13 deficeincy
TTP
What is ITP treatable with, and what should you def not treat it with?
monoclonal antibodies- DO NOT treat with platelet transfusion
What are the types of immunotherapy for Hodgkin’s lymphoma
Checkpoint inhibitors and monoclonal antiboidies (CD30)
What are treatments for low grade non-hodgkin lymphoma
OBSERVATION or local radiation and chemotherapy.
What are treatments for high grade non hodgkin lymphoma
-Treatment is URGENT
- R-CHOP
- Rutuxin
- Radiation
-SCT or CAR-T
When is HgbF replaced by HbgA
6 months of age
In people who are heterozygous for sickled cell anemia, what is the Hgb level
Normal
Basophillic stippling is associated with what disorder and is caused by what pathology
Related to lead toxicity
Caused by ribosomal granules in the RBC, strange ribosomal granules.
What are the 2 noteable key symptoms of Vitamin B 12 deficiency
Glossitis and Neurological symptoms
What value measures the range of variation of RBC volume
RDW
Higher= greater variation in size
If you have a anisocytosis, what is your RDW value
high because there is less uniformity between cells
4 types of normocytic, normochromic anemia
- Acute blood loss
- Aplastic anemia
- Leukemias
- Bone marrow infiltration
4 types of microcytic, hypochromic anemia
-Iron deficiency
- thalassemia
- lead poisioning
- anemia of chronic disease
2 types of macrocytic, normochromic anemia
- Folate deficiency
-B12/pernicious anemia
If you have your Hct, what do you do to get your RBCS?
Multiply by 100k
Hct * 100,000= RBCs
How to get Hb from RBC
RBC * 3 = Hb
Hb to Hct calculation
Hb*3= Hct
What are platelets released from
Released from megakaryocytes in bone marrow
If they are released improperly, they might go into blood as large platelets.
ITP has LARGE platelets
TTP has LARGE multinucleated platelets
On a blood smear, a normal sized RBC is about how big
the diameter of the nucleus of a small lymphocyte
Spherocytes are seen in which 2 diseases
Autoimmune hemolytic anemia
Hereditary spherocytosis
Dacryocytes are seen in which disease
primary myelofibrosis
Schistocytes are seen in which disorders
Thallasemias
Hemolytic anemia
TTP
DIC
How do you differentiate ITP from TTP on a smear
TTP- schistocytes and multinucleated platelets
ITP- large platelets (Thrombocytopenia). ALSO smudge cells possibly on ITP b/c you could have a luekemia
Target cells are seen in which disorders
Hemoglobin C disese
Iron deficiency
Thalassemia
RBCs that have been ripped or torn against fibrin strands or thrombi in circulation, or have been buffeted against unyielding structure in macrocirculation. Fragments reseal and persist in circulation and are highly significant.
Schistocytes
Fragments seen in severe burns, DIC, TTP, and hemolytic processes
Fine, medium, or coarse blue granules with uniform distribution within the red blood cell
Basophillic stipling
Abnormal Ribosomal RNA degredation
Lead poisioning, Thalassemias, alcoholism
Which disorder is associated with increased indirect bilirubin
Hemolysis (Sickle cell, hereditary spherocytosis, G6PD, Autoimmune hemolytic, TTP, HUS, DIC)
Ineffective erythropoiesis (Thalassemias, B12 or Folate deficiency, MDS)
Which test/value improves faster than anemia symptoms do?
LDH- elevatted LDH is a sign of increased cell turnover (not any specific kind of cell). RBCs that stop being hemolyzed as quickly will have less LDH over time
How many classes of Igs
5
Which antibody makes up 85% of serum Igs and predominates late immune responses
IgG
Only Ig to cross plecenta
What is the only Ig to cross the placenta
IgG
Which Ig predominates immune responses early on
IgM
Characteristics of IgM
- 3rd most abundent in serum
- Heavy chain is Mu
-primary response - Short life, so presence means recent infection
- Opsonization, complement fixation, agglutination
Which Ig’s heavy chain is Mu
IgM
If IgM is in the serum of a newborn, what dose this indicate?
Congenital infections
IgM cannot cross plecenta
So, syphilis, toxoplasmosis, etc, is indicated
Which Ig has the epsilon heavy chain
IgE
Infectious mononucleosis will cause an increase in which Ig
IgM (polyclonal)
Immunosuppresive chemotherapy will cause a decrease in which Ig
IgG
What are the triad of symptoms in hemolytic transfusion reaction
- Fever
- Flank pain
- Red/Brown urine
Define Urticaria
HIVES
Proteins in blood react with IgE
Must pause the transfusion, but if you only have urticaria, resume after giving the patient an antihistamine
Does Taco or trali have a fever or hyper/hypotension
TRALI- lung injury has Fever and HYPO tension
Taco has an O and HypOtension has an O. Each get one.
Does Hodgkins or Non hodgkins have spread from lymph nodes
Non Hodgkins spreads all over and to GI/Skin– does NOT stay in one place
Bone pain with lytic lesions from a monoclonal protein is indicative of what
Multiple myeloma
Monoclonal protein spike, bence jones proteinuria
What do you give your patient if they have neutropenia
GCSF
Describe febrile neutropenia
Fever >38 measured 2x 1 hour apart or 38.5 at any time
moniter vitals closely to make sure not septic
see where infecction is coming from (pneumonia, UTI)
Gram - bacteria are harmed so new bacteria come in and invade
Manage with vanco and linezolid
What is the most common oncologic emergency
Septic shock
What does septic shock arise from
Neutropenic fever
If you have a myeloid leukemia, what are the cells implicated
Myeloid cells- basophils, eosinophils, neutrophils, RBCs, platelets
Aml- myeloblasts
CML- basophils, eosinophils, neutrophils
If you have lymphoid leukemia, what cells are implicated
Lymphoid stem cell
Lymphoblast for ALL
B lymphocyte, T lymphocyte, NK for CLL
ALL PE signs
ALL= Anemic, Lumpy (lymphatonopathy, splenomegaly), Limping (MSK pain)
CBC findings on ALL
Everything (ALL) are odd (Pancytopenia)
Anemia, thrombocytopenia, WBCs
Bone marrow biopsy is key- Lymphoblasts
***Severely impaired bone marrow fx- no increased retics
Treatment of ALL
Chemo (Steroids, Vincristine, anthracycline)
CAR-T or allogenic bone marrow transplant (IF RELAPSE)
In ALL, what is retic count
LOW- Severely impaired bone marrow.
Which disorder has M3 in it
AML has APL (M3 variant)
AML risk factors
Benzene exposure
AML PE
Gingival hyperplasia
How long do you have to live with APL if not treated, and how do you treat it?
Have <1 month to live
With treatment, very high cure rate
All-Trans retinoic acid to treat
Chemotherapy
Transplant
3 steps to treatment in AML/ALL
- Induction
- Consolidation
- Maitnence
Factor V leiden, Hemophillia A, and Hemophilia B all have what similar value on a coag pannel
All have increased PTT, normal PT, and normal platelets
Mild cases of hemophilia are treated with what
DDAVp- desmopressin- increases factors