Hematology Flashcards
Best initial test when concern for anemia
CBC with peripheral smear
What are supplementary tests to order on CCS when concerned about anemia?
Reticulocyte count, Iron labs, TSH and T4, haptoglobin, bilirubin, LDH, B12/folate
What type of anemia is caused by blood loss?
Iron deficiency
What are iron labs in iron deficiency anemia?
Iron: Low FERRITIN: LOW TIBC: High Transferrin saturation: Low Elevated RDW
Most accurate test to dx IDA
Bone marrow biopsy (do not order on CCS)
Tx for IDA
Oral ferrous sulfate
Iron studies in anemia of chronic disease
Iron: Low
FERRITIN: HIGH
TIBC: Low
Transferrin saturation: Low or normal
Buzzword
Microcytic anemia with target cells
Thalassemia
Most accurate test for dx of thalassemia
Hemoglobin electrophoresis
(Beta form: elevated HgA2 and HgF)
(Alpha form: normal)
Is sideroblastic anemia microcytic or normocytic?
What are potential causes (3)?
Microcytic
Alcohol, Isoniazid, Lead exposure
Iron levels in sideroblastic anemia are ….
High
Most accurate test for dx of sideroblastic anemia
Prussian blue stain
Alpha thalassemia is most accurately diagnosed by …
DNA sequencing
What is one of the only anemias with a high RDW and why?
IDA
As there is less iron the RBCs made are smaller and smaller which leads to large variation in size
Besides fatigue what else does B12 deficiency anemia present with?
Peripheral neuropathy but can be other neurologic problems like dementia
Buzzwords
Glossitis and diarrhea associated anemia
B12 deficiency
Buzzwords
Hypersegmented neutrophils and oval cells
Macrocytic anemia
What happens to reticulocyte count in B12 deficiency anemia?
Low
B12 deficiency causes destruction of cells just as they leave bone marrow which causes low reticulocyte count
What happens to homocysteine and methylmalonic acid levels in B12 and folate deficiency?
B12: homocysteine and methylmalonic acid levels rise
Folate: homocysteine levels rise
You’ve confirmed a low B12 and high methylmalonic acid. What is the next test you should run for etiology?
Antiparietal cell Abs and anti-intrinisic factor Abs to look for pernicious anemia
Hemolytic anemia labs
Indirect bili, reticulocyte count, LDH, haptoglobin, PBS
Indirect bilirubin: High Reticulocyte count: High LDH: High Haptoglobin: Low Spherocytes on PBS
What should you order for the PE on CCS for sickle cell anemia?
Complete physical exam
What are best initial therapies to offer a patient presenting with sickle cell anemia pain?
Oxygen, pain meds, fluid hydration
Is fever in sickle cell anemia a emergency?
What medication should you give?
Yes
Levofloxacin, moxifloxacin, or ceftriaxone
What should be repleted in all sickle cell patients?
Folate
Most accurate test for parvovirus B19 dx in sickle cell patients
Parvovirus DNA PCR
Parvovirus infection in sickle cell is best treated how?
Transfusions and IVIG treatment
At discharge what should sickle cell patients be discharged on?
Folate
Hydroxyurea
Pneumococcal vaccination
What is the only manifestation of hemoglobin SC disease?
Renal: hematuria, UTI, isosthenuria (inability to concentrate urine)
Most accurate diagnostic test for autoimmune hemolysis
Coomb’s test
Best initial therapy for autoimmune hemolysis
Steroids (prednisone)
If there are recurrent episodes of hemolysis in autoimmune hemolysis then what is the most effective therapy?
Splenectomy
Are ‘warm Abs’ IgM or IgG?
IgG
*Only IgG abs respond to steroids and splenectomy
Buzzword
Mycoplasma or EBV associated anemia
Cold-induced hemolysis
What will be the result of a standard Coomb’s test in cold-induced hemolysis? What test will be positive?
Negative
Complement test will be positive
How is cold-induced hemolysis treated?
Rituximab
*No response to steroids, splenectomy, or IVIG
Is cold-induced hemolysis IgG or IgM?
IgM
What is mode of inheritance of G6PD deficiency?
X-linked recessive
What are common triggers of G6PD deficiency anemia?
Infection
Oxidizing drugs (e.g. sulfas, primaquine, dapsone)
Fava beans
Buzzwords
Heinz bodies and bite cells are seen in this anemia
G6PD deficiency
What is the most accurate test for dx of G6PD deficiency and when should it be ran?
G6PD level but should be done 2 months after the episode. If done too early it will be normal bc the most deficient cells will have been destroyed
Recurrent episodes of hemolysis, splenomegaly, bilirubin gallstones, and a high MCHC are signs of which anemia cause?
Hereditary spherocytosis
What is the most accurate diagnostic test for hereditary spherocytosis?
Osmotic fragility test
Tx of hereditary spherocytosis
Splenectomy
Buzzword
E. Coli 0157:H7
Hemolytic-Uremic Syndrome
Ticlodipine can predispose to what macroangiopathic hemolytic anemia?
Thrombotic thrombocytopenic purpura (TTP)
Findings in HUS
Intravascular hemolysis
Elevated BUN and creatinine
Thrombocytopenia
Findings in TTP (5)
Intravasacular hemolysis Elevated BUN and creatinine Thrombocytopenia Fever Neurological abnormalities
ADAMTS-13 level in TTP is …
Low
Should you transfuse platelets in a patient with TTP or HUS?
No
Severe cases of TTP or HUS are treated with what?
Plasmapharesis
Patient presents with pancytopenia and dark urine only in am. Dx?
Paroxysmal nocturnal hemoglobinuria
What is most common cause of death in paroxysmal nocturnal hemoglobinuria?
Large vessel thrombosis
Most accurate test for diagnosis of PNH
CD55 or CD59 Ab (aka decay accelerating factor)
Best initial tx of PNH
Steroids
*Transfusion-dependent patients with severe disease are treated with eculizumab
What are some drugs which may cause methemoglobinemia?
Nitroglycerin, amyl nitrate, nitroprusside dapsone, -caines
What may cause SOB with clear lungs on exam, normal CXR, and brown blood? What is tx?
Methemoglobinemia
Methylene blue
A patient receiving blood transfusion and becoming anaphylactic with normal LDH and bilirubin is caused by …
IgA deficiency
Delayed jaundice in a patient receiving a blood transfusion is caused by …
Minor blood grouping incompatability
Buzzwords
Auer rods associated with
AML
Buzzwords
DIC associated leukemia
M3, acute promyelocytic leukemia
Best initial test in diagnosis of acute leukemia
PBS showing blasts
What is the most important prognostic indicator in acute leukemia which may be an indication for bone marrow transplantation in addition to chemo?
Cytogenetic abnormalities suggesting high rate of relapse
Chemo agents used in acute leukemia are?
What is added in M3 (acute promyelocytic) leukemia?
What is added for ALL?
Idarubicin (or daunorubicin) and cytosine arabinoside
In M3 add all-trans retinoic acid
In ALL add intrathecal MTX
In patients with extremely high WBC count in leukemia what is the recommended therapy?
Leukapheresis and hydroxyurea to reduce WBC count
What presents with pancytopenia, elevated MCV, low reticulocytes, and macroovalocytes?
Myelodysplasia
Are there more or less blasts in myelodysplasia compared to leukemia?
Less. Myelodysplasia is like a preleukemia syndrome which is slower and may ultimately progress to leukemia
Buzzword
Pelger-Huet cells association
Myelodysplasia
*The cell is a neutrophil with two lobes
What is the most common cause of death in patients with myelodysplasia?
Infection or bleeding
Tx of myelodysplasia
Supportive with transfusions as needed
*Azacitadine (increases survival), decitabine, and lenalidomide
Why do patients have early satiety in CML and CLL?
Splenomegaly may compress stomach
What happens to the following values in CML?
Leukocyte alkaline phosphatase (LAP)
Neutrophils
LAP decreases
Neutrophils increase
Elevated LAP score indicates …
Leukomoid reaction (reactive increase in WBC)
Buzzword
Philadelphia chromosome
bcr/abl in CML responding to Gleevac (imatinib)
Best initial therapy CML
Imatinib
*BM transplantation cures CML but is never the best initial test
Best initial test for CLL
PBS showing lymphocyte smudge cells
Tx for CLL
Stage based. Early forms of disease do not need treatment.
Advanced stage disease: fludarabine (prolongs survival), rituximab, and cyclophosphamide
Should you treat elevated WBC count (asymptomatic) in patients with CLL?
No
If fludarabine fails in CLLthen what med is used?
Alemtuzumab (anti-CD52 agent)
Smear showing hairy cells and immunophenotyping are the most accurate tests/findings for what disease?
Hairy cell leukemia
Best initial therapy in hairy cell leukemia
Cladribine (2-CDA)
How can myelofibrosis and hairy cell leukemia be distinguished?
Hairy cell leukemia has elevated TRAP
Normal TRAP in myelofibrosis
Buzzword
Tear drop cells
Myelofibrosis
What mutation is seen in myelofibrosis?
JAK2
*Ruxolitinib inhibits JAK2 and is occasionally used
Best initial therapy for myelofibrosis
Lenalidomide or thalidomide
Headache, blurred vision, dizziness, and pruritis may be seen in what disorder?
Polycythemia vera
MCV in polycythemia vera
Low
JAK2, normal O2 sat, and low MCV are seen in …
Polycythemia vera
Best initial therapy for polycythemia vera
Phlebotomy
Meds used for polycythemia vera and essential thrombocythemia
Hydroxyurea (lowers cell count)
Aspirin
Anagrelide (if thrombocythemia)
What is erythromelalgia?
Pain in hands and feet seen in erythrocythemia from high platelet count
What are most common causes of death of multiple myeloma?
Renal failure and infection
Elevated levels of monoclonal IgG antibody on SPEP may suggest what disease?
Multiple myeloma
Buzzwords
Bence-Jones protein on UPEP
Multiple myeloma
How will a peeripheral smear appear in multiple myeloma?
Rouleaux formation from RBC clumping in chains
What is a prognostic indicator in multiple myeloma?
Beta-2 microglobulin
What medicine reverses the renal dysfunction in multiple myeloma?
Bortezomib
B12 and LAP levels are changed how in polycythemia vera?
Both elevated
What is the most specific test for diagnosis of multiple myeloma?
Bone marrow biopsy
Tx of multiple myeloma
Melphalan and steroids
Thalidomide, bortezomib, and lenalidomide may be added
Patients that are relatively young (
Autologous stem cell BM transplantation
Patients with hypercalcemia in multiple myeloma should be managed how?
Hydration and diuresis
Asymptomatic elevation of IgG on SPEP
Monoclonal gammopathy of unknown significance
What are the best initial tests in Waldenstrom’s Macroglobulinemia?
Serum viscosity test
SPEP with elevated IgM
Best initial therapy for symptomatic patients with Waldenstrom’s
Plasmapharesis
additional tx are rituximab fludarabine, chlorambucil, or rituximab
Chronic Hep B and C can cause …
Aplastic anemia
What are therapy options for patients with aplastic anemia?
If young and a match BM transplant. If not either then antithymocyte globulin and cyclsporine
How are Hodgkin’s disease and Non-Hodgkins’s disease different in terms of disease localization?
Hodgkin’s: Starts centrally, near cervical region and spreads out from center
Non-Hodgkin’s: Widespread
What is the best initial test for diagnosing Hodgkin’s and NHL?
Excisional lymph node biopsy
Histologically how do Hodgkin’s and NHL differ
Hodgkin’s has Reed-Sternberg cells (look like owls)
Describe the staging scheme for lymphoma
Stage I: Single lymph node group
Stage II: Two lymph node groups on one side of diaphragm
Stage III: Lymph node groups on both sides of diaphragm
Stage IV: Widespread disease
What other tests are helpful in staging lymphoma?
CXR
BM biopsy
CT scan with contrast
How are the different stages of lymphoma treated in general terms (e.g. radiation, chemo, etc)?
Stages I and II: Radiotherapy and chemo
Stage III and IV: Chemo
Tx for Hodgkin’s Disease
ADVB
Adriamycin, bleomycin, vinblastine, dacarbazine
Tx for NHL
CHOP Cyclophosphamide Hydroxyadriamycin Oncovin (vincristine) Prednisone
If anti-CD20 positive then add Rituximab
Von Willenbrand’s Disease is caused by what abnormality? What happens to platelet and clotting times?
Platelet dysfunction from lack of vWF
Platelet counts are normal
PTT may be elevated because vWF stabilizes factor VIII
What are the most accurate tests for diagnosing vWD?
Ristcetin cofactor assay (can tell if vWF is working even if normal)
vWF level
What is first-line treatment for vWD?
Desmopression (DDAVP)
If DDAVP does not work for vWD then what is another treatment option?
Factor VIII replacement (contains both vWD and factor VIII)
Antibodies to what receptor cause ITP?
Antibodies to glycoprotein IIb/IIIa receptor
Peripheral smear demonstrating large platelets and BM showing megakaryocytes may be indicative of what disorder?
ITP
If ITP is suspected what is the best next step?
Provide prednisone. It is a diagnosis of exclusion and time should not be spent trying to diagnosis
In severe ITP presenting low platelet count (e.g.
Provide IVIG
What do romiplostim and eltrombopag do?
They’re thrombopoietin analogs which directly stimulate megakaryocytes and can be used to treat chronic ITP
Normal ristocetin and vWF levels in a patient with platelet-type bleeding and kidney failure indicates what problem?
Uremia-induced platelet dysfunction
What is specifically disrupted in platelets from patients with uremia-induced platelet dysfunction?
Their ability to degranulate
Tx of uremia-induced platelet dysfunction
DDAVP, dialysis, estrogen
What is treatment for factor XII deficiency?
No treamtent needed
What is treatment for factor XI deficiency?
Fresh frozen plasma
Does thrombosis in HIT occur more in veins or arteries?
Veins
Best initial diagnostic tests for HIT
Platelet factor 4 Abs or heparin-induced platelet antibodies
Buzzword
Russel viper venom test
Most accurate test for lupus anticoagulant
What are clotting times in antiphospholipid syndrome and other unique lab results?
Elevated aPTT
Normal PT
False positive VDRL
Tx of APAS
Heparin followed by warfarin
How does protein C deficiency present when starting anticoagulation?
Skin necrosis when patients go on warfarin
Venous thrombosis
What is treatment of protein c deficiency?
Heparin followed by warfarin
What is MOA of heparin?
Inactivates thrombin and activated Xa
