PANCE Prep- Hematology Flashcards
___ presents in adults with a cell lineage of the immature granulocytic cells seen in the peripheral blood e.g. myeloblasts, promyelocytes.
-Auer rods also are commonly seen in this condition
Acute myelogenous leukemia (AML)
When a patient receives more than one total blood volume (10 units) without replacement of ___, the patient will continue to bleed without administration of ___
clotting factors
fresh frozen plasma
A positive direct Coombs’ test may be seen in what condition
Autoimmune hemolytic anemia
hereditary spherocytosis has negative coombs
Hereditary spherocytosis is detected utilizing ___
- the osmotic fragility test
2. negative Cooombs
___ is indicated in Coumadin overdosage
Fresh frozen plasma
___ is indicated in type 2 and 3 patients with von Willebrand disease bleeding
___ is indicated in type 1 von Willebrand disease bleeding
vWF concentrate
DDAVP** (vasopressin) used to bleeding
__ is indicated for patients with Hemophilia A.
Factor VIII or cryoprecipitate
Once the diagnosis of heparin induced thrombocytopenia is suspected treatment should include:
- immediate discontinuation of all forms of heparin
2. treatment with a direct thrombin inhibitor should begin
___ is used for heparin overdosage
Protamine sulfate
What condition is caused by a disorder in the red blood cell membrane?
Hereditary spherocytosis
What Rh genotype of the mother and fetus puts the baby at risk for hemolytic disease of the newborn?
Mother: Rh negative
Fetus: Rh positive
Low molecular weight heparin (LMWH) dosage is based on which of the following?
Weight
___ is the diagnostic study of choice for G6PD deficiency.
G6PD assay
___ is indicated in the evaluation of hemolytic anemia
Hemoglobin electrophoresis
An African American male is placed on hydroxychlorquine (Plaquenil) for travel to Africa. Within six weeks he presents complaining of fatigue. CBC shows anemia. Which of the following is the diagnostic study of choice in this patient?
G6PD assay
CML (chronic myeloid leukemia) patients typically present with what physical exam finding
splenomegaly
Hereditary spherocytosis presents with what type of anemia
a normocytic, normochromic anemia and many spherocytes.
___ presents with a prolonged aPTT and normal platelet count and function.
Hemophilia A
An 18 year-old woman presents to the clinic complaining of fatigue. She reports a past history of lifelong frequent nosebleeds and bleeding gums. She also has menorrhagia. Her mother and maternal grandfather have a similar bleeding history. Initial lab results are as follows: WBC 9,500/mm3, Hgb 10.9 g/dL, HCT 33%, MCV 69 fL, MCHC 26 pg and platelets 284,000/mm3. Which of the following tests should be ordered to evaluate this patient’s diagnosis?
Bleeding time and platelet aggregometry
*presentation is consistent with a congential qualitative platelet disorder, most likely von Willebrand’s Disease, necessitating a bleeding time and evaluation of platelet function.
What therapies is recommended for a 13 month-old child with sickle cell disease?
Folic acid and penicillin V
*Ferrous sulfate is not globally recommended for patients with sickle cell disease.
What Leukemia is most closely associated with the Philadelphia chromosome?
chronic myelogenous leukemia
___ is used to treat immune-mediated hemolytic anemias
Prednisone
Splenectomy- long term definitive tx
immune-mediated hemolytic anemias presents with ___ red blood cells.
normocytic, normochromic
___ is characterized by bleeding from many sites as all coagulation factors are consumed and then broken down, leading to:
- decreased fibrinogen level and platelet count,
- prolonged PT and PTT
- presence of FIBRIN SPLIT products.
Disseminated intravascular coagulation
____ transports Fe throughout the body. This carrier can be measured indirectly by ____
transferrin
TIBC
Describe transferrin, TIBC and ferritin levels in iron deficiency anemia and anemia of chronic disease
Fe deficiency- Increased transferrin, increased TIBC, decreased ferritin
anemia of chronic disease- Decreased transferrin, decreased TIBC, and increased ferritin
*both have decreased serum Fe
HgbS on hemoglobin electrophoresis
sickle cell anemia (hemolytic)
Microcytic anemia with normal/increased serum Fe or no response to Fe tx
Thalassemia
How to differentiate alpha and beta thalassemia
alpha: hemoglobin electrophoresis: normal Hgb ratios of HbgA, A2, and F
beta: hemoglobin electrophoresis: DECREASED HgbA, INCREASED HgbF, increased HgbA2,
G6PD is associated with what?
- sulfa drugs (plaqunil, UTI meds- Bactrim)
- fava beans
- infections
TTP Pentad
- thrombocytopenia
- hemolytic anemia
- kidney damage
- Neurologic sx*
- Fever*
HUS Triad
- thrombocytopenia
- hemolytic anemia
- kidney damage* (more so than TTP)
- Rouleaux Formation
- RBCs stick together like “stack of coins”
- Increased ESR
- high protein
- Multiple Myeloma
- Infections
Bite cells (degmacytes) are seen with:
thalassemia, G6PD
Schistocytes (fragmented RBCS) are seen w/
Hemolytic anemias (DIC, TTP, HUS)
Keratocytes (helmet shaped RBCs) are seen w/
TTP, HUS, DIC, prosthetic valves
Echinocytes “Burr cells” are seen w/
Uremia
Hypersegmented neutrophils are seen w/
B12** and folate deficiencies
Presentation:
- *neurological sx, paresthesias (stocking and glove-like paresthesia), gait abnormalities, memory loss, dementia (acute or permanent)
- diarrhea, glossitis, anorexia
- macrocytic anemia
B12 (cobalamin) deficiency
TX: IM B12- watch for signs of HYPOKALEMIA
How do you dx B12 and folate deficiency
- Peripheral smear: MCV >115, hypersegmented neutrophils
- increased homocysteine, increased methylmalonic acid*
- Schilling test
*normal methymalonic acid with folate def.
Causes of macrocytic anemia
- B12 (cobalamin) def.
- Folate def.
- ETOH abuse
- liver disease
- hypothyroidism
Causes of microcytic anemia
- iron def.
- alpha/beta thalassemia
- early anemia of chronic disease (ACD)
- lead poisoning
- sideroblastic anemia
Presentation:
- pagophagia, pica, angular cheilitis, koilonychia
- microcytic, hypochromic anemia
Iron def. anemia
TX: ferrous sulfate 325mg orally daily
- Vit. C increases Fe absorption
- see increase in reticulocyte count w/in 7 days
dysphagia + esophageal webs + atrophic glossitiis + Fe def.
Plummer-Vinson syndrome
How do you dx Fe def. anemia
Decreased ferritin, increased TIBC, decreased serum FE
- Increased RDW*,
- decreased transferrin saturation <15%
Presentation:
abdominal pain w/ constipation, neurologic symptoms (ataxia, fatigue, learning disabilities, coma, shock, metabolic acidosis
-basophilic stippling and ringed sideroblasts
lead poisoning anemia (plumbism)
How do you dx lead poisoning anemia (plumbism)
Peripheral smear: microcytic, hypochromic anemia w/ BASOPHILIC STIPPLING and RINGED SIDEROBLASTS
- Increased serum lead and serum Fe,
- Xray: “lead lines”
Normally after ___ old, adult Hgb is predominant Hgb produced
6 months (HgbA- 2 alpha and 2 betas)
Think ___ if microcytic anemia w/ normal/increased serum Fe or no response to Fe tx
Thalassemia
How do you dx thalassemia
- hypochromic, microcytic anemia w/ normal Fe
- Perpherial smear: TARGET cells, teardrop cells
- HEINZ bodies in HgbH disease
- Hgb Electrophoresis**
G6PD affects most commonly in
primarily AA males
how do you dx sickle cell disease
decreased Hgb, HCT and increased reticuolocytes
-sickled erythrocytes +/- Howell-Jolly bodies**
Hgb electrophoresis*
Tx of sickle cell disease
- pain control: IV hydration and oxygen in pain crisis
- Hydroxyurea: reduces freq. of pain crises
- Folic acid
- immunize for S. pneumococcus, H. influena and N. meningoccus *SHiN)
Common presentations of sickle cell disease
- Dactylitits MC 1st presentation at 6-9 months
- Osteomyelitis (esp. Salmonella)
- Functional asplenia
Dark cola-colored urine during the nigh tor early AM w/ partial clearing during the day
-thrombosis ex. hepatic, mesenteric
paroxysmal nocturnal hemoglobinuria
DX: flow cytometry best screening test*
TX: Eculizumab
Primary hemostasis involves __
Secondary hemostasis involves __
Primary hemostasis- platelets form a plug at site of vascular injury: platelet adhesion activation and aggregation
Secondary hemostasis- clotting factors respond in a cascade to form fibrin strands which strengthens the platelet plug
What does PT and PTT measure and give examples of things that prolong them
PT: EXTRINSIC factor pathway: (7 and 10**)
ex. heparin, DIC< vWD, hemophilia A and B
PTT: INTRINSIC: (8, 9, 11, 12**)
ex. warfarin, Vit K def, DIC
heparin OD antidote:
warfarin OD antidote:
heparin: protamine sulfate
warfarin: vit K
Decreased AdamTS12
primary TTP
tx: plasmapheresis
Seen predominately in children (usually preceded by E. coli O157:H7, shigella or salmonella gastroenteritis:
-thrombocytopenia, microangiopathic hemolytic anemia, kidney failure (uremia)
HUS (hemolytic uremic syndrome)
TX: observation / IV fluids to maintain renal perfusion
- plasmapheresis if severe
- *Antibiotics worsen the condition
Etiologies of DIC
- infections (gram neg. sepsis MC)
- malignanices
- Obstetrics
- massive tissue injury
how do you DX DIC
- Increased thrombin, decreased fibrinogen, increased PTT/PT/INR,
- increased d-dimer (increased fibrinolysis)
TX: DIC
tx underlying cause
2. FFP if severe bleeding
- BIMODAL peaks at 20 and again at 50y/o
- Associated with EBV- MC in males
- PAINLESS lymphadenopathy but may develop painful lymph nodes w/ ALCOHOL
Hodgkin Lymphoma
DX: biposy: REED-STERNBERG cell pathognomoic**
mediastinal lymphadenopathy
TX: radiation +/- chemo
**highly curable compared to NHL
- PAINLESS lymphadenopathy peripheral MC- extranodal sites common: GI, skin, CNS MC
- abdominal pain
Non-hodgkin lymphoma
tx: unpredictable course
- follicular: Rituximab: ab vs CD20 on B cells
- Diffuse large B cell: chemo
Describe presentation of multiple myeloma
“Bones BREAK” or CRAB
-(Ca elevated, Renal failure, Anemia, Bone osteolytic lesions)
Bone pain: osteolytic, destructive lesion
Recurrent infections from leukopenia
Elevated Calcium (hypercalcemia)
Anemia
Kidney failure (Bence Jones proteins in urine)
DX and tx of multiple myeloma
DX:
- serum protein electrophoresis: monoclonal (M) protein spike
- urine protein electrophoresis: Bence-Jones proteins*
- CBC: ROULEAUX formation and increased ESR
- Skull radiograph: punched-out lytic lesions
- **BM biopsy: plasmacytosis <10%
TX: autologous stem cell transplant is definitive tx +/- chemo
MC childhood malignancy and common with Downs syndrome
Fever MC sx
CNS sx, hepatosplenomegaly, lymphadenopathy
ALL
DX: BM: hypercellular w/ >20% blasts
TX: combo chemo
MC leukemia in adults
- fatigue, painful lymphadenopathy
- SMUDGE cells on peripheral smear
CLL (B cell)
TX: observe if indolent, Fludarabine chemo if progressive
lab abnormalities and tx of tumor lysis syndrome
hyperuricemia, hyperkalemia, hypocalcemia, hyper phosphatemia, acute renal failure
allopurinol, IV fluid
What leukemia is associated with Philadelphia chromosome
CML (Philadelphia CreaM cheese)
Overproduction of all 3 myeloid cell lines- lymphocyte line normal
- JAK2 mutation
- hypervisocity or thrombosis
- Splenomegaly,
Polycythemia vera (primary erythrocytosis)
increased iron, ferritin, and transferrin
- liver dysfunction
- HF, cardiomyopathy, arrhythmias
- hypogonadism
- pancreatic insufficiency
- Metallic or Bronze skin– bronze diabetes
Hereditary hemochromatosis (excessive iron deposition in heart, liver, pancreas and endocrine organs)
DX: liver biopsy is gold standard– hemosiderin
tx: phlebotomy
MC inherited caused of hypercoagulability
Factor V leiden
MC clotting disorders
Factor V leiden
Protein C def.
Antithrombin III Def.
Pernicious anemia is due to deficiency in what
intrinsic factor (secreted by parietal cells)
MC cause of hypercalcemia in malignancy
Increased production of parathyroid hormone-related peptide (PTHrP)
*MC CA- lung, breast CA and myeloma
2nd MC cause is osteolytic metastasis (NOT OSTEOBLASTIC)
PMH: miscariages
presents w/ clot, lacy erthematous rash on extremities
Antiphosopholipid antibody syndrome
DX: anticardiolipid antibodies, lupus anticoagulant, and anti-beta 2 glycoproteins
Presentation:
- 70y/o pt.
- CBC: leukocytosis and lymphocytosis (usually found incidentally on ROUTINE bloodwork)
- 1/3 develop autoimmune hemolytic anemia, and 5% develop ITP
Chronic lymphocytic leukemia (B Cell)
*presents with a WBC count greater than 20,000/microliter and absolute lymphocyte count of greater than 5000/microliter
Spontaneous hemarthroses are pathognomonic for:
Hemophilia A and B
What is the most serious delayed toxicity that is seen w/ the anthracycline drug class?
cardiomyopathy
Bleomycin used in Hodgkin’s lymphoma and testicular CA has the known toxicity of ___
Pulmonary toxicity
Common findings and tx of Primary polycythemia
- Elevated Hgb and HCT
- Elevated platelet- thrombosis/hypervisocity
- Splenomegaly
- facial plethora (flushed face*)
- JAK2 mutation
- engorged retinal veins
TX: phlebotomy
(diuretic use is suggestive of spurious PV)
4 main common types of myeloproliferative neoplasms
- polycythemia vera (PV)
- essential thromboytosis
- CML
- primary idiopathic myelofibrosis
Presentation:
- Bleeding gums, epistaxis
- NO splenomegaly
- Hx of URI
- Purpura or petechiae
- Low platelets
Immune thrombocytopenia purpura (ITP)
TX: steroids***** +/- IVIG
*transfusion or splenectomy if platelet count drops below 5,000-10,000
TX in Adults: steroids or Rituxan and Eltrombopag are approved for ADULTS ONLY
If left untreated, Vit. B12 def. can cause
Irreversible neuropathy
Vit. B12 is produced by ___ and absorbed in ___
parietal cells
terminal ileum
- Decreased WBC, RBCS and platelets (pancytopenia)
- BM biopsy: hypoplastic marrow, NO blasts
- Causes: radiation exposure, chemical exposure to pesticides or solvents, viruses, paroxysmal nocturnal hemoglobinuria and sulfa compounds
aplastic anemia
*if farmer patient think aplastic anemia
How do you dx Von Willebrand’s Disease
- S/S: Mucocutaneous bleeding: easy bruising, epistaxis, gums GI, menorrhagia
- decreased vWF levels
- +/- prolonged PTT
* ** PTT prolongation worsen w/ aspirin - Decreased ristocetin activity test gold standard**
MC bleeding disorder- inherited autosomal dominant
TX: Vasopressin or DDAVP
Presentation:
platelet count starts to drop after being on coumadin and heparin and start developing thromboses
Heparin-induced thrombocytopenia (HIT)
*caused by autoantibodies of platelet factor 4 (PF4)
Myelodysplastic Syndrome can progress to ___ in select patients
acute leukemia
Packed red cell transfusions (PRBCs) can cause temporary ___
Risk of iron overload has been recognized after packed red blood cell transfusion of __ units
HYPERkalemia
more than 20 units
__ products carry the highest risk of tranfusion-related acute lung injury (TRALI) among all blood products
plasma, apheresis platelets, and whole blood
TX: oxygen and possibly ventilation
*occurs w/in 6 hours of transfusion (presentation: hypoxia, pulmonary infiltrates, hypotension, cyanosis)
One unit of Packed red cell transfusions (PRBCs) is expected to raise the hemoglobin ___
1 Hgb
The following peripheral smears match what diseases?
- Target Cells
- Howell-Jolly bodies
- Heinz Bodies
- Rouleaux formation
- Poikilocytosis
- Bence Jones protein in urine
- Target Cells: liver dz, hemoglobinopathy like thalassemia
- Howell-Jolly bodies: post-splenectomy, splenic dysfunction, sickle cell
- Heinz Bodies: G6PD
- Rouleaux formation (stacked coin appearance of RBCS): myeloma
- Poikilocytosis: extreme iron deficiency
- Multiple myeloma
Presentation:
- Hx of RA
- Splenomegaly and neutropenia
Felty’s syndrome
DX: Clinical, CBC, +/- BM biopsy to r/o other causes
- Prolonged PTT
- Mixing study of blood to normal serum results in persistently prolonged PTT
- lupus anticoagulant
- Factor 8 inhibitors
- Contamination w/ heparin
- PTT stays prolonged= presence of an inhibitor
- if PTT corrects w/ mixing study= deficiency of one of the factors (12, 11, 9, 8)
What med is the best choice in patients on chemotherapy to help minimize the risk of neutropenia
Pegfilgrastim (long acting colonystimulating factory (CSF) support)
Sickle cell disease (HgbS) is what type of inherited disease?
autosomal dominant
*genetic counseling and testing should be performed if fhx even if healthy could have sickle cell trait
Most common etiologies of Iron deficiency anemia
- CHRONIC blood loss (not acute)
2. Pregnancy- bc of increased production of erythrocytes
When using tamoxifen in the treatment of malignancy, one must keep what toxicity in mind?
secondary malignancy- increased risk of uterine cancer
What tumor marker can be used in the screening of patients for cancer?
alpha-fetoprotein- used to screen for hepatocellular carcinoma
(screen in those w/ chronic hep B, C and cirrhosis)
*PSA, CEA, CA 19-9 are not sensitive or recommended
What is Christmas disease
Hemophilia B (factor 4) -X-linked recessive disease affecting primarily males
Vitamin K deficiency occurs primarily with ___
primary biliary cirrhosis
what is the most common cause of hypercalcemia in malignancy?
osteoblastic lesions from bone metastasis
does Hodgkin’s lymphoma or non-Hodgkin’s lymphoma have Reed-Sternberg cells
Hodgkin’s
Hodgkin’s lymphoma has what type of age distribution?
There is also an increased incidence of it after exposure to ___
bimodal
EBV
Evaluation of ___ can suggest myelodysplastic syndrome (MDS) but ___ is diagnostic
peripheral smear
BM biopsy
Transfusion related acute lung injury (TRALI) is rare but potentially fatal reaction to blood product infusions that occurs within __ hours of transfusion. Even though TRALI can happen w/ any blood product, _____ have the greatest risk.
6 hours
plasma blood products, apheresis platelet concentrates and whole blood (rarely used today)
Risk of iron overload has been recognized after packed red blood cell transfusions of more than __ units
20
packed red blood cell transfusions are known to cause ___ electrolyte disturbance
hyperkalemia (from leakage of K+ out of the RBCs)
Name the peripheral smear match with the disease:
- Target cell
- Howell-Jolly bodies:
- Heinz Bodies:
- Rouleaux formation:
- Poikilocytosis:
- Target cell: liver dz, hemoglobinopathy (thalassemia,)
- Howell-Jolly bodies: s/p splenectomy or asplenic pt
- Heinz Bodies: G6PD deficiency
- Rouleaux formation: elevated plasma proteins ie. myeloma
- Poikilocytosis: Extreme iron deficiency
PTT is sensitive to what factors?
XII, XI, IX, VIII
When mixing a pts prolonged PTT blood w/ 1:1 ratio w/ normal donors blood what does it mean if:
- PTT corrects:
- stays prolonged:
- corrects= def. of one of the factors (8, 9, 11, 12)
2. prolonged= a presence of an inhibitor (ie. lupus anticoagulant, factor VIII inhibitors, contamination w/ heparin)
