Exam 1 studying Flashcards by Morgan Evans

What may occur when correcting folate or B12 deficiency?

Hypokalemia

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True or false: Elevated indirect bilirubin and LDH may be seen with hemolytic anemia

True

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Gold standard for identifying immune cause (e.g., warm/cold autoantibodies) of hemolytic anemia is what?

Direct antiglobulin (Coombs) test (DAT)

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True or false: Intrinsic hemolytic anemias are usually hereditary

True

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Autoimmune Hemolytic Anemia (AIHA):
1) Is it inherited or acquired?
2) What is seen?

1) Acquired
2) “warm” agglutinins = IgG autoantibodies that avidly bind at body temperature
“cold” agglutinins = IgM autoantibodies that avidly bind at colder temperatures (e.g., exacerbated by cold exposure)

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Metformin
Histamine 2 receptor antagonists (H2 Blockers)/proton pump inhibitors (PPIs) [decreased gastric acidity]
Nitrous oxide (NO)

These drugs may interfere with the absorption of what?

B12

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Phenytoin, sulfasalazine, trimethoprim-sulfamethoxazole may decrease absorption of what?

Folate

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True or false: Exfoliative dermatitis may increase folate requirement

True

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Jaundice
Dark urine (hemoglobinuria)
Cholelithiasis (chronic hemolysis)
Extramedullary hematopoiesis
Hepatosplenomegaly

These are all Sx of what?

Hemolytic Anemia

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“Bite” cells or “blister” cells AND Heinz bodies may be seen in what cause of anemia?

G6PD deficiency

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Hydroxyurea (500–750 mg orally daily) is mainstay of treatment of what?

Sickle cell anemia

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Paroxysmal nocturnal hemoglobinuria (PNH) is a cause of what?

Intrinsic hemolytic anemia (membrane defects)

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Fluoroquinolones
Nitrofurantoin
Phenazopyridine
Antimalarials (primaquine, tafenoquine)
Sulfonylureas (glipizide, glyburide)

These all may trigger what?

G6PD episode

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Elliptocytes = elliptical-shaped RBCs, ranging from oval to cigar-shaped.

What may cause these?

Hereditary elliptocytosis

A type of intrinsic hemolytic anemia (membrane defects)

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Hemoglobinopathies are an example of what?

Intrinsic RBC defects (hemolytic anemias)

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Give 3 examples of hemoglobinopathies

Sickle cell
Thalassemias
Unstable Hgb variants

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Give an example of a Autosomal recessive anemia

Sickle cell

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If a patient has unexplained neonatal jaundice what should you suspect (besides physiologic jaundice)?

G6PD

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A sickle cell aplastic crisis is usually associated with viral or other infection or _____________ deficiency

folic acid

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HbS is a lab finding in what?

Sickle cell (trait 40%, anemia up to 98%)

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Diagnosis of sickle cell anemia is confirmed by what test?

Hemoglobin electrophoresis

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What ethnic group is more likely to have alpha thalassemia?

southeast Asia and China

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Cooley’s anemia and Mediterranean anemia are 2 names for what?

Beta thalassemia major

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Differentiate between the 3 severities of thalassemias

Thalassemia trait:
Lab features without significant clinical impact
Thalassemia intermedia:
Occasional RBC transfusion requirement or other moderate clinical impact
Thalassemia major:
Disorder is life-threatening and transfusion-dependent  most eventually die from iron overload due to RBC transfusions

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Basophilic stippling is seen in which thalassemia?

Beta

Mediterranean origin (Italian, Greek) are more likely to have what kind of thalassemia?

Beta-thalassemia

In _________ thalassemia, there's a deletion, and in ________ thalassemia, there's a mutation

alpha; beta

of Alpha-Globin Genes Transcribed, list the names of the conditions -No genes -1 gene -2 genes -3 genes -4 genes

-0 genes: Thalassemia Major (Hydrops fetalis) -1 gene: Hemoglobin H disease (Intermedia) (beta4) -2 genes: Thalassemia Trait (Minor) -3 genes: Silent carrier (Minima) -4 genes: Normal

MCV is strikingly low (60–75 fL) despite the modest anemia in what condition?

Alpha Thalassemia Trait (Minor):

A patient Heterozygous for beta thalassemia has what?

Beta Thalassemia Minor (Trait)

For Beta thalassemia major, what is the treatment of choice and the only available cure?

Allogeneic stem cell transplantation

What is caused by point mutations?

Beta thalassemias + Sickle hemoglobin (Hb S)

Do you give folate for alpha thalassemia, beta thalassemia, or both?

Both

Compared to iron deficiency anemia*, patients with thalassemia have what lab findings?

Lower MCV Normal or elevated RBC count (rather than low) More abnormal peripheral smear at modest levels of anemia Usually a reticulocytosis Iron studies are normal or have elevated transferrin saturation and/or ferritin

Elevated levels of Hgb A2 and F would indicate what?

Beta thalassemia

Plummer-Vinson Syndrome (webbing of the esophagus causing dysphagia) is associated with what type of anemia?

Iron deficiency

Severely hypochromic cells Target cells (bulls-eye appearance) Pencil-shaped or cigar-shaped cells These can all be Sx of what?

Severe iron deficiency anemia

What 2 values may be high with ACD/AI?

1) Ferritin (iron stores): high or normal 2) Inflammatory Markers (CRP and/or ESR): high or normal

JAK2 mutation causes a condition that causes itching after bathing. What is this condition?

Polycythemia vera (PV)

Does primary or secondary polycythemia involve increased EPO?

Secondary

In which form of polycythemia will you see increased RBCs, increased leukocytes, and increased thrombocytes?

Primary

What is the most common cause of neutropenia?

Drug reaction

What type of polycythemia is commonly from diuretics, vomiting, diarrhea?

Hemoconcentration polycythemia

What contains all coagulation factors and fibrinogen, and needs to be ABO typed?

FFP

What factors are in cryoprecipitate?

Factors 8 & 13, von Willebrand factor, fibrinogen, and fibronectin.

Hypofibrinogenemia is an indication for what type of transfusion?

Cryoprecipitate

Blood products can be contaminated with bacteria (especially platelets – cannot be refrigerated): What will G+ cause? What abt G-?

Gram+ may cause fever and bacteremia Gram- (endotoxin) may cause septic shock, DIC, AKI

True or false: Hgb rises by expected amount despite leukoagglutinin reactions

True

Ineffective erythropoiesis in beta thalassemia, sideroblastic anemia, or other inherited anemias can cause what?

Iron overload

Most causes of HH are homozygous for what variant?

C282Y variant

What is classically mediated by Shiga toxin?

HUS

List some X linked recessive conditions

1) G6PD deficiency 2) Hemophilia A 3) Hemophilia B

Differentiate Desmopressin (DDAVP) and Emicizumab

1) Desmopressin (DDAVP): factor 8 and von Willebrand factor 2) Emicizumab: brings activated factor IX and factor X together, effectively replacing the cofactor function of factor VIII in the clotting cascade

The oral dose of Vit K is what?

5–10 mg/day

_____ becomes prolonged prior to ________ because Vit. K deficiency first affects the activity of factor 7, whose turnover is most rapid

PT; PTT

Mucocutaneous bleeding, ecchymoses and petechiae, and isolated thrombocytopenia are the main Sx of what?

Immune Thrombocytopenic Purpura (ITP)

Name 2 types of Thrombotic Microangiopathy (TMA):

Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS)

Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) have what 2 main findings?

Thrombocytopenia Microangiopathic hemolytic anemia (MAHA)

ADAMTS-13 inhibitor can be detected in what?

Acquired TTP

Fever Anemia Thrombocytopenia Renal dysfunction Neurologic abnormalities These are the main findings of what?

TTP

MAHA can present as what?

Schistocytes + Elevated LDH/indirect bilirubin, decreased haptoglobin

True or false: Platelet transfusion is contraindicated in TMAs

True

True or false: schistocytes may be seen in DIC

True

List the DIC labs

-Progressive thrombocytopenia -Prolongation of PT and aPTT -Low/declining fibrinogen -Elevated D-dimer levels (produced when the blood clots dissolve) -May have schistocytes on blood smear (MAHA) -HELLP (“hemolysis, elevated liver enzymes, low platelets”): includes elevated AST/ALT and kidney injury

True or false: Both Protein C and protein S are vit K dependent

True

Venous Thromboembolism (VTE) is the major manifestation of what?

Factor V Leiden

VwF deficiency type __________ can resemble hemophilia A

Hemophilia C is autosomal recessive and most common in what group?

Ashkenazi Jews

Half of pts with MDS are asymptomatic, but what are the common Sx?

fatigue (anemia), infection (neutropenia), or bleeding (thrombocytopenia) (Possible splenomegaly on exam)

The only curative therapy for MDS is what?

Allogeneic stem cell transplantation

True or false: MDS is an ultimately fatal disease. Explain

True Death mostly due to infections or bleeding Some forms have favorable 5-year survival rates >90%

Possible gingival hypertrophy/stomatitis & rectal fissures occurs with what?

acute monocytic leukemia – a subtype of AML seen more commonly in children

BCR::ABL fusion gene in blood an bone marrow is a hallmark of what?

CML (Philadelphia chromosome)

Imatinib (a Tyrosine kinase inhibitor/ TKI) is the Tx of choice for what?

CML

What type of cancer affects the B lymphocytes?

CLL

Auer rods are strongly associated with what?

AML

Usually present with painless lymphadenopathy (peripheral or central) Indolent lymphomas usually disseminated at diagnosis This describes what condition?

NHL

Very high WBCs (150K+) and a left shift in myeloid cells are signs of what?

CML

What is the hallmark of AML?

Pancytopenia with circulating blasts (usually >20% in BM)

When does CML turn into AML?

When it becomes >20% blasts

One of the only leukemias not linked to radiation exposure (and is mostly familial) is what?

CLL

Is lymphadenopathy with CLL painful?

Non-painful

Which type of cancer has a bimodal age distribution? (hint: HIV is also a risk factor)

Hodgkin lymphoma

What is the hallmark lab finding of CLL?

isolated lymphocytosis

Pruritus and severe pain following alcohol ingestion are Sx of what? (hint: has continuous spread)

Hodgkin lymphoma

Flow cytometry shows coexpression of CD19, CD5 on lymphocytes in what condition?

CLL

Reed-Sternberg cells on lymph node biopsy indicate what?

Hodgkin lymphomas

What type of cells are these?

Reed-Sternberg cells

Organ transplant recipients and those w autoimmune conditions are at risk for what?

NHL

True or false: you should Tx an 85 y/o with HL with curative intent

True

What are "B symptoms"? (as seen in HL)

fevers, night sweats, and/or weight loss

What type of cancer affects all age groups?

NHL

Monoclonal immunoglobulin (paraprotein) formation is a main Sx of what?

Plasma cell myeloma (PCM)

Plasma cell myeloma is most common in what demographic?

Older african americans

What 2 conditions are assoc with agent orange?

CLL + Plasma cell myeloma (PCM)

Most common presenting symptoms related to anemia, bone pain, kidney disease, & infection Bone pain most common in back, hips, ribs, proximal long bones Can present as pathologic fracture (common in femoral neck & vertebrae) Spinal cord compression (plasmacytoma) This describes what?

PCM (Plasma cell myeloma)

Anemia is nearly universal in a condition where Rouleax formation of RBCs is common. What is this condition?

Plasma cell myeloma

Most common paraprotein of PCM on SPEP/UPEP or IFE is what?

IgG

List the mnemonic for myeloma (PCM) defining events

CRAB HyperCalcemia (Total calcium >11.0 mg/dL) Renal injury (creatinine >2 mg/dL) Anemia (Hgb <10 g/dL) Bone disease (lytic lesions on x-ray, MRI, PET/CT)

Descr. diagnosis of PCM/MM vs. smoldering vs. MGUS

1) PCM: ≥10% clonal plasma cells in the bone marrow + a CRAB 2) Smoldering: ≥10% BM clonal plasma cells but no “myeloma-defining events 3) MGUS: <10% BM clonal plasma cells

What type of cancer is most common in hispanics?

CML

B12 deficiency (but not folate deficiency) causes accumulation of what?

MMA

True or false: Homocysteine is elevated in either B12 or folate deficiency

True

Name an autoimmune cause of B12 deficiency that should be treated indefinitely

Pernicious anemia (PA) (use anti-IF antibody testing)

Cobalamin is also called what?

Vitamin B12

Describe Vitamin B12 (Cobalamin) Supplementation

1) Injections: 1 mg (1,000 mcg) injection usually daily for first week, weekly for next month, and then monthly (in lieu of monthly injections, can alternatively transition to daily oral supplementation) 2) Oral or SL : 1-2 mg/d

Females without other neural-tube defect (NTD) risks (i.e., average risk) should take a daily prenatal multivitamin containing folate ______ mg starting at least 1 month prior to conception and continuing through pregnancy

0.4

What is the gold standard for diagnosing aplastic anemia?

bone marrow aspiration & biopsy

Exam 1 studying Flashcards

(108 cards)