Hematology/Oncology Flashcards by Taylor Davis

In what organelle (within erythrocyte) does heme synthesis take place

Mitochondria

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What deficiency is associated with warfarin skin necrosis

Protein C/ S deficiency- warfarin inhibits these factors since dependent on vitamin K and get a hypercoagulable state, this leads to microvascular occlusion and hemorrhagic skin necrosis ( Factor 7 and protein C have the shortest half lives)

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Benefits of COX 2 inhibitors vs. having COX 1 inhibition?

COX 2 inhibition have potent anti-inflammatory with out GI ulceration side effects, COX 1: inhibits platelet aggregation and can lead to GI ulceration

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How are myoglobin and hemoglobin related

Structurally analogous, myoglobin is not sigmoid and would be similar to 4 beta monomers—> most left shift curve possible and myoglobin is momomeric

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What features are associated with hair cell leukemia?

Dry tap (unsuccessful bone marrow aspiration), lymphocytes with cytoplasmic projections, TRAP positive stain

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What are the atypical cells seen on blood smear in EBV infection?

CD8+ T cells, although EBV preferentially infects B cells

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What does von Willebrand factor bind to?

Platelet glycoproteins (Gp1b) and collagen

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What is dactylitis and what disease is it associated with?

Painful swelling of hands and feet, Sickle cell disease

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What does haptoglobin do?

Binds circulating hemoglobin and reduced free renal excretion of free hemoglobin preventing tubular injury

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5 Ps Acute intermittent porphyria and what enzyme is deficient and what is treatment?

Painful abdomen
Port wine urine
Polyneuropathy
Psychological disturbances 
Precipitated by drugs ( alcohol, starvation)

Porphobilinogen deaminase

Tx: glucose and heme which INHIBIT ALA-synthase (rate limiting step)

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What is extramedullary hematopoiesis and what are bone marrow signs?

Presence of erythroid precursors in liver and spleen, EPO hyperplastic marrow

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Atypical venous thrombosis in adults <50, which no aPTT response after giving protein C

20% FACTOR V Leiden—> factor V resistance to protein C so—> hypercoagulable

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Heme oxygenase

( found in macrophages) Converts heme into biliverdin( green color after bruise), CO, ferrous iron and then bilverdin is broken down by biliverdin reductase to yellow bilirubin which is then transported by albumin to liver

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Burkitt lymphoma

t(8;14), cMYC overexpression=transcription activator

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Mantle cell lymphoma

t(11;14), cyclin D1 overexpression=promoter of G1–>S phase

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Follicular lymphoma

t(14;18), BCL2 overexpression=INHIB apoptosis

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Chronic myelogneous leukemia (CML)

t(9;22), ABL gene from chr 9–> INC tyrosine kinase activity

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Polycythemia vera (describe dz and associated mutation with function)

Myeloproliferative disorder characterized by UNCONTROLLED RBC production, mutation in JAK2 ( cytoplasmic tyrosine kinase)= hematopoietic cells more sensitive to growth factors, also see: splenomegaly, aquagenic pruritus(histamine from basophils), facial plethora, gout (inc cell turnover)

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Aplastic anemia triad

Low hemoglobin, thrombocytopenia, ABSENT hematopoietic stem cells in bone marrow( high lipid content), would see and INC in EPO if also have normal renal function

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How can you treat hemophilia?

Give thrombin because no factor 8 or 9 to convert factor 10 into factor 2 (thrombin)

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CD 14 is a cell marker for and what disease can it be seen in that has caseating granulomas

Monocyte macrophage lineage with pale pink granular cytoplasm and secondary TB

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Why is amyloid associated with multiple myeloma?

Forms from accumulation of mono colonial immunoglobulin light chains

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What enzyme deficiency can present similar to glucose 6 phosphate dehydrogenase deficiency

Glutathione reductase deficiency: if don’t have, can’t use NADPH to reduce glutathione to get rid of free rads

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Basophillic stippling and microcytic hypochromic anemia are associated with

Lead poisoning ( can also see abdominal pain and blue lead lines in teeth)

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B cells mature in the _____ and undergo what process there

Bone marrow, VDJ recombination

What process are B cells undergoing in the follicles

Isotype switching and requires interaction of CD40 receptors on B cells with CD40 ligand of activated T cells (CD154)

Peru ate kinase deficiency leads to ______ anemia. How does the spleen react to this

Hemolytic, red pulp hyperplasia because has to work overtime to get rid of lysed RBCs

The anal canal drains to which lymph nodes

Superficial inguinal

Lymph from superior bladder drains into

External iliac nodes

Lymph from prostate drains into

Internal iliac nodes

Lymph from upper 1/3 rectum drains into

Inferior mesenteric nodes

Myeloblasts stain positive for

Peroxidase

Sickling in sickle cell disease is promoted by situations with ______ oxygen, ______ acidity, _____ blood volume, _____ levels of 2,3 bisphosphoglycerate

Low oxygen, increased acidity, low blood volume, increased

Patients who receive more than 5-6 L of blood transfusion have high levels of _____ in the blood and this can chelate ____ and leads to parathesias

Citrate, calcium

What is the translocation associated with APML and how do you treat?

15;17 15: PML 17: RARA Tx with all trans retinoic acid

Methemoglobinemia happens because _____ cause poisoning by inducing the conversion of oxidized Fe3+ state. The _____ of oxygen remains unchanged

Nitrates, partial pressure (amount of oxygen dissolved in the plasma)

Inhibition of proteasomes may lead to an _____ of proapoptotic proteins

Excess

G6PD deficiency can lead to hemolytic anemia. What conversion is inhibited

Glucose 6 phosphate to 6 phosphogluconate

Hemoglobin F does not bind to 2,3 bisphosphoglycerate and as a result has a _____ affinity for O2 than Hemoglobin A

higher, this allows HbF to extract lots of oxygen from mom

Hemoglobin S leads to ______ interactions that cause sickling

hydrophobic, glu switched for a valine (nonpolar)

Clinical features of back pain at night, not relieved by rest or analgesics, advanced age, and systemic symptoms suggest what kind of cause

metastasis to bone

DIC is associated with sepsis and how does it affect aPTT and PT, platelets, fibrinogen

``` BOTH PROLONGED (ONLY ONE THAT does this when compared to ITP and TTP) -dec platelets, dec fibrinogen ```

The key growth factors for angiogenesis are?

VEGF: vascular endothelial growth factor, fibroblast growth factor

The ________ in basement membranes poses a physical barrier to the sprouting of new blood vessels

laminin

In a post menopausal women with microcytic anemia, need to rule out ________

occult blood loss (Ex: from GI malignancy)

Renal cortical cells sense hypoxia and respond by synthesizing and releasing _______. This leads to an elevated hematocrit

EPO, esp in sleep apnea

how does uremic platelet dysfunction affect PT, aPTT, bleeding time, platelet count?

platelet count=dec, everything else normal

Pure red cell aplasia is associated with ______, lymphocytic leukemia, parvovirus B19

thymomas and have preserved myeloid and megakaryotic elements

Factor V Leiden is a defect in ______ and predisposes you to _______ events

protein C, thrombotic

_______ defiiciency is a megaloblastic anemia that can occur in weeks in alcholics

folate

PT is affected by _____ and if levels are really low sometimes vitamin K supplementation still won't help because not enough around to be activated

factor 7

bony pain in and older man who smokes is ______ cancer

prostate

how does vwF disease affect PTT, bleeding time, PT

PTT: prolonged, PT: normal, bleeding time: prlonged

_________ is the most common indolent non-Hodgkin lymphoma in adults. It typically has a waxing and waning course

follicular lymphoma

Tumor cells detach from neighboring cells with dec expression of

E-cadherin

Tumor cells adhere to the basement membrane which is facilitated by inc expression of

laminin

Tumor cells invade the basement membrane with inc expression of

proteolytic enzymes (metalloproteinases, cathepsin D protease)

The major chronic pathologic changes in the spleen of patients with sickle cell results from?

microvessel occulsion -this leads to a fibrotic, shrunken, brown (excess hemosiderosis from extravascular hemolysis of RBCs)

________ is defined by a hematocrit leve >52% in men and 48% in women

erythrocytosis

Erythrocytosis with and increase in RBC mass indicates

absolute erythrocytosis

Erythrocytosis with a normal RBC mass indicates

relative erythrocytosis

Primary erythrocytosis is associated with ____ levels and is caused by

low EPO, myeloproliferative disorders (like polycythemia vera)

Secondary erythrocytosis is associated with ___ levels and is caused by

high EPO, chronic hypoxia (COPD, high altitude, smoking)

Activating mutations of the KRAS gene lead to constuitive activation of the _________ pathway leading to?

epidermal growth receptor pathway (EGFR), increased cell proliferation and growth -tumors with KRAS mutation are resistant to anti EGFR drugs

Large eosinophillic casts with Bence Jones proteins and a pt. w/ fatigue, back pain, elevated serum protein, and azotemia lead to?

multiple myeloma

SLE induced antiphospholipid syndrome is made by the presence of _________. How does this affect PTT and why? Will it be corrected with FFP?

lupus anticoagulant, prolongs PTT because it is an antiphospholipid antibody and it binds the reagent to the test, no

Which cancer is associated with auer rods and what's the translocation

APML, t(15;17)

What precursor ALL has TdT+, CD 10, CD19

precursor B-ALL

What precursor ALL has TdT+, CD 2,3,4,5,7,8

precursor T-ALL

__-ALL is more likely to present with a large anterior mediastinal mass that can compress the great vessels, causing _________. It can also compress the esophagus/trachea leading to

T, superior vena cava syndrome (edema of face and arms), dysphagia, inspiratory stridor/dyspnea

___ thalasemia minor is common in people of Mediterranean descent. You can see inc level of which hemoglobin? What is the initial step in pathogenesis of this disorder?

beta, HbA2: b/c dec beta chain synthesis. mRNA of the beta globin gene is disrupted

Patients with sickle cell disease have inc requirement of what vitamin and what can this lead to?

folic acid (b/c of inc erythrocyte turnover), macrocytic anemia

If you have a low reticulocyte count that signifies a _____ response to anemia and lack of ________

inadequate, erythropoiesis

The most common for DIC in pregnancy is release of ___________ from an injured placenta into maternal circulation

tissue factor, DIC presents with bleeding from incision sites, and IV sites from MASSIVE consumption of platelets and clotting factors

Dysplasia is a _______ change in epithelial cells

reversible, once it invades BM=carcinoma and not reversible

Schistocytes can occur from mechanical trauma such as damage with ______

prosthetic valves

What does haptoglobin do? When do levels decrease?

binds free Hgb and promotes its uptake by the reticuloendothelial system, when lots of free Hgb is released into the serum such as in intravascular hemolysis

Paroxysmal nocturnal hemoglobinuria has an associated deficiency of which two CD proteins? It is associated with _______ anemia and _______thrombosis. What is its clinical triad? How does this affect the kidney

CD55/59. aplastic, hepatic vein, - hypercoagulability, pancytopenia,hemolytic anemia - hemosiderosis in the kidney from chronic hemolysis with breakdown of iron containing erythrocytes

Migratory ______ is suggestive for visceral cancer. Why does hypercoagulability develop?

thrombophlebitis, b.c adenocarcinomas produce a thromboplastin like substance capable of causing intravascular coagulations

How are these levels in multiple myeloma: RBCs, platelets, Ca, ESR, urine protein, serum protein, serum albumin

- dec RBCs and platelets(b/c plasma cells infiltrate the bone marrow) - elevated Ca and ESR( systemic inflammation and proteolytic bone lesions) - inc urine and serum protein but DEC serum albumin (b/c plasma cells make M protein--> rouleaux formation (stacks of RBCs) so get an inc in total SERUM protein but albumin typically dec

The gene for heavy chain of Igs is found on which chromosome?

_______calcemia is a common finding after blood transfusions and why? What are symptoms?

Hypocalcemia, Citrate in the blood will bind calcium Vague muscle weakness, irritability

Diffuse B cell lymphoma has cells that are ____ positive

CD5, presents with B symptoms

In sickle cell trait, only _____ of hgb is abnormal. And what is a symptom they might have?

35-40%, episodic hematuria (sickling of RBCs in the renal medulla from thromboembolic events)

Newborn born at homewith umbilical stump bleeding probably has a _______ deficiency

Vitamin K, so only PT and aPTT will be prolonged -baby didn’t get vitamin K shot in the hospital

von willibrand disease leads to ____ PTT and ____ bleeding time

prolonged, prolonged | vWF is a cofactor for factor 8 as well

sporadic forms of _______ lymphoma can present with highly mitotic, basophilic lymphocytes surrounding clear zones of macrophages. most common presentation in north america is:

burkitt lymphoma, constipation, abdominal mass( enlarged LNs)

What is WAGR syndrome?

Wilms tumor, Aniridia(partial or complete lack of the iris), Genital abnormalities, mental Retardation

unfractionated heparin is better at blocking ____ than regular heaprin

thrombin | -both affect factor Xa but unfractionated can also attack Xa AND thrombin

Rat poison has ______ in it so if someone overdoses good to treat with

warfarin, FFP to replace clotting factors

Cryoprecipitate has what factors

8,13,vWF,fibrinogen

Eculizimab blocks the conversion of ______

C5 and MAC complex

What is somatic hypermnutation

component of affinity maturation, its a randomized mutation process of variable regions, then follicular dendritic cells present antigen and B cells that prod the antibodies with highest affinity are positively selected

________ presents as hypoparathyroidism, adrenal insufficiency, chronic mucocutaneous candidiasis, skin dystrophy

AIRE gene dysfunction

________ presents with Bierbeck granules, painful bone lytic lesions, eczematous rash. Cells are ____ positive and express _____ protein

LangerHans histiocytosis, CD1a, S100

Hemolytic disease of the newborn typically results from ________

erythrocyte opsonization from maternal antibodies (hydros fetalis)

Mutations that cause production of hemoglobin with higher oxygen affinity ____ the ability of Hgb to release oxygen to the peripheral tissues so get a _______

compensatory erythrocytosis that helps maintain oxygen delivery

Hereditary spehrocytosis results in ____ membrane stability -->______ erythrocyte osmotic fragility

dec, inc

Sickle cell can cause _______ induced hemolysis due to Hgb S polymerizing and causing damage

hypoxia

Red blood cell fragments of burr and helmet cells are significant for

mechanical trauma | -look for "just got a heart valve"

isoniazid inhibits pyroxidine phosphokinase--> ___ deficiency. This is a cofactor for __________ and when dec can lead to ________anemia

B6, ALA synthase, sideroblastic

Lysing of RBCs in glycerol situation w/ inc osmotic fragility leads you to think

hereditary spherocytosis

__________ is a mutation in the HFE gene and typically interacts with the _______ receptor that acts as a sensor for iron stores. They're at inc risk of developing __________ and _________

primary hemochromatosis, transferrin - this causes receptors to detect falsely low levels - hepatocellular carcinoma, liver failure

Integrins interact with the ECM by binding to which 3 proteins?

collagen, fibronectin, laminin

reticulocytes contain a bluish cytoplasm and why is it blue?

because of ribosomal RNA

_____ and ______ cause a WBC>50,000 with an inc in___________. How is the leukocyte (neutrophil) alkaline phosphatase leveL?

CML, leukemia reaction - precursor forms( bands, metamyelocytes, myelocytes) - CML: Dec (b/c WMCs are cytochemically abnormal), leukemoid rxn: inc

Phosphorylated Rb leads to _____ transcription

inc

erythroblastalis fetalis can lead to high levels of unconjugated bilirubin and SEVERE consequence of this is

kernicterus (can damage basal ganglia, thalamus)

Hereditary spherocytosis can lead to __________ unconjugated bilirubin--->

inc, gallstones

reverse anticoagulation (heparin use) with

protamine sulfate

replace warfarin overdose with

fresh frozen plasma

reverse low fibrinogen levels with

cryoprecipitate (fibrinogen, factor 7, vWF)

heparin doesn't cross the placenta because its _____phillic

hydro (high water solubility)

What are three hematological abnormalities in PNH?

- hemolytic anemia (C5b-9 MAC formation), - hypercoagulable state (complement attack on platelets--> release of thrombogenic molecules - pancytopenia (aplastic anemia from lack of CD55,59)

________ are the only drugs used to treat brain tumors

nitrosureas

CA 19-9 is for ______ cancer

pancreatic

CA-125 is for _______ cancer

ovarian/endometrial cancer

abnormally large platelets on peripheral blood smear

Bernard Soulier (absence GP lb)

von willi brand disease you will see ___ PT, _____ platelet count, _____ PTT, ______ bleeding time

normal PT, normal platelet count, prolonged PTT (factor 8), prolonged bleeding time

Hematology/Oncology Flashcards

(119 cards)