HemeOnc First Aid Flashcards by Lauren Sophiea

What do RBC membranes contain?

Cl-/HCO3- antiporter; allows export of HCO3 and transport CO2 from periphery to lungs

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What granules do thromboyctes have and what do they contain?

dense granules
- ADP
- Ca2+
alpha granules
- vWF
- fibrinogen
- fibronectin

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vWF receptor is:

Fibrinogen receptor is:

GpIb

GpIIb/IIIa

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What do specific granules of neutrophils contain?

leukocyte alkaline phosphatase (LAP), collagenase, lysozyme, and lactoferrin

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what do azurophilic granules (lysosomes) of neutrophils contain?

proteinases, acid phosphatase, myeloperoxidase, and B-glucuronidase

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Hypersegmented neutrophils are seen in

Vit B12/folate deficiency

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increased number of band cells reflect states of

increased myeloid proliferation (bacterial infections, CML)

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found in the blood, this cell has a large, kidney shaped nucleus and extensive “frosted glass” cytoplasm

monocyte

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What role do macrophages have in precipitating septic shock?

Lipid A from bacterial LPS binds CD14 on macrophages to initiate septic shock

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causes of eosinophilia include

NAACP

Neoplasia
Asthma
Allergic process
Chronic adrenal insufficiency
Parasites (invasive)

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What do basophils contain?

heparin and histamine

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basophilia is uncommon but can be a sign of

myeloproliferative disease, esp CML

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What do mast cells release once they bind the Fc portion of IgE and IgE crosslinks?

histamine, heparin, tryptase, and eosinophil chemotactic factors

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What do dendritic cells express on their surface? What are these cells called in the skin?

MHC class II and Fc receptors

Langerhans cells in the skin

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Once mature, B cells migrate to peripheral tissues including

follicles of lymph nodes, white pulp of spleen, and unencapsulated lymphoid tissue

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All T cells have what CD marker?

CD28 (costimulatory signal) necessary for T cell activation

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What cells have “clock-face” chromatin distribution and eccentric nucleus, abundant RER, and well-developed Golgi?

plasma cell

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vWF is from where?

Weibel-Palade bodies of endothelial cells and alpha-granules of platelets

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What drugs inhibit GpIIb/IIIa directly?

abciximab, eptifibatide, and tirofiban

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What activates vWF to bind GpIb?

failure of aggregation of this is seen in…

ristocetin

von Willebrand disease and Bernard-Soulier syndrome

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what does an acanthocyte look like and what pathology is associated with it?

spur cell

Liver disease, abetalipoproteinemia (states of cholesterol dysregulation)

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What is a dacrocyte and when would I see it?

teardrop cell

bone marrow infiltration (eg myelofibrosis); mechanically squeezed out of its home in the bone marrow

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What is the official name of a bite cell?

Degmacyte

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When would I see echinocytes?

burr cells

ESRD, liver disease, pyruvate kinase deficiency

projections more uniform than acanthocyte

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When do you see target cells?

HbC disease, Asplenia, Liver disease, and thalassemia

4 alpha globin allele deletions

Hb Barts - gamma4 hydrops fetalis

3 alpha-globin allele deletions

HbH (beta 4)

secondary hemochromatosis, "crew cut" on skull XR, "chipmunk facies", increased risk of Parvovirus B19

Beta-thalassemia major

lead inhibits ferrochelatase and ALA dehydratase leading to

decreased heme synthesis and increased RBC protoporphyrin

Ssx of lead poisoning?

* Lead lines on gingivae (Burton lines) and on metaphyses of long bones on XR * Encephalopathy and erythrocyte basophilic stippling * Abdominal colic and sideroblastic anemia * Drops - foot and wrist

What is used for lead chelation in kids?

succimer

genetic cause of sideroblastic anemia is

Xlinked defect in ALA synthase gene

How do you differentiate orotic aciduria from ornithine transcarbamylase deficiency?

both will have increase orotic acid, but ornithine transcarbamylase deficiency will also have hyperammonemia

in orotic aciduria, what is the defect in?

defect in UMP synthase with inability to convert orotic acid to UMP (de novo pyrimidine synthesis pathway)

Historically, how was vit B12 deficiency diagnosed?

diagnosed with Schilling test, a 4 stage test that determines if the cause is dietary insufficiency v malabsorption

Nonmegaloblastic macrocytic anemia is likely caused by... RBCs still have macrocytosis but without

alcoholism, liver disease hypersegmented neutrophils

rapid onset of anemia within first year of life due to intrinsic defect in erythroid progenitor cells dx? what is a major physcial feature of this person?

Diamond Blackfan anemia triphalangeal thumbs

notable causes of intravascular hemolysis

mechanical hemolysis paroxysmal nocturnal hemoglobinuria microangiopathic hemolytic anemias

hemolytic anemia in a newborn

pyruvate dehydrogenase deficiency AR, decrease ATP, rigid RBCs, extravascular hemolysis increase 2,3BPG leads to decreased hemoglobin affinity for O2

coombs negative hemolytic anemia, pancytopenia, and venous thrombosis

paroxysmal nocturnal hemoglobinuria

What labs will you see with PNH?

CD55/59 negative RBCs on flow cytometry

Porphyria cutanea tarda condition affects what enzyme?

uroporphyrinogen decarboxylase (AD mutation)

Prophobilinogen deaminase, previously known as uroprophyrinogen I synthase has an autosomal dominant mutation in what condition?

acute intermittent porphyria

acute intermittent porphyria has what symptoms? How is this treated?

* painful abdomen * Port-wine colored urine * Polyneuropathy * Psychological disturbances * Precipitated by drugs (P450 inducers, alcohol, starvation) tx with glucose and heme, which inhibit ALA synthase

PT tests function of what factors?

common and extrinsic pathway (I, II, V, VII, X)

PTT tests function of what factors?

common and intrinsic pathway (all factors except VII and XIII)

In a mixing study, where normal plasma is added to a pt's plasma, how do you know if you have a clotting factor deficiency v factor inhibitor?

clotting factor deficiencies should correct (PT or PTT returns to normal) whereas factor inhibitors will not correct

hemophilia A, B, and C are deficiencies of what factors? What are their inheritence patterns?

A - factor VIII (X linked recessive) B - factor IX (X linked recessive) C - factor XI (autosomal recessive)

What will vitamin K deficiency do to PT, PTT and bleeding time?

increase both PT and PTT bleeding time is normal

inhibition or deficiency of ADAMTS 13 causes

Thrombotic thrombocytopenic purpura decreased degradation of vWF multimers; increased platelet adhesion, increased platelet aggregation and thrombosis

Anti-GpIIb/IIIa antibodies are seen in

immune thrombocytopenia

defect in platelet integrin alpha IIB beta3 (GpIIb/IIIa) leading to defect in platelet-to-platelet aggregation, and therefore platelet plug formation normal platelet count

Glanzmann thrombasthenia

Defect in platelet plug formation causing large platelets, decreased GpIb (Defect of platelet to vWF adhesion)

Bernard-Soulier Syndrome

tx for von Willebrand disease?

desmopressin, which releases vWF stored in endothelium

What are complications of Factor V Leiden?

DVT, cerebral vein thromboses, recurrent pregnancy loss

thrombotic skin necrosis with hemorrhage after administration with warfarin, I am thinking...

pt has protein C deficiency

What does cryoprecipitate have in it?

fibrinogen, factor VIII, factor XIII, vWF, and fibronectin

Why is there a risk of hyperkalemia with blood transfusions?

RBCs may lyse in old blood units and then get transfused with their contents everywhere

What are the four types of Hodgkin lymphoma?

* nodular sclerosis * lymphocyte rich * mixed cellularity * lymphocyte depleted

Genetics of Burkitt lymphoma

t(8:14) c-myc (8) and heavy chain Ig(14)

genetics of diffuse large B cell lymphoma

alterations in Bcl-2, Bcl-6 | (MC type NHL in adults)

genetics of follicular lymphoma

t(14:18) heavy chain Ig (14) and BCL-2 (18)

presents with painless "waxing and waning" lymphadenopathy

follicular lymphoma

genetics of mantle cell lymphoma

t(11:14) adult males - cyclin D1 (11) and heavy chain Ig (14), CD5+

marginal zone lymphoma genetics and association with

t(11;18) cyclin D1 (11) and BCL-2 (18) a/w chronic inflammation (eg Sjogren syndrome, chronic gastritis/MALT lymphoma)

In this AIDS-defining illness, with variable presentation, mass lesions on MRI need to be distinguished from toxoplasmosis via CSF analysis or other lab tests

Primary Central Nervous System Lymphoma

atypical CD4+ cells with "cerebriform" nuclei and intraepidermal neoplastic cell aggregates (Pautrier microabscesses)

mycosis fungoides/Sezary syndrome (T cell leukemia)

ssx of multiple myeloma

CRAB * hyperCalcemia * Renal involvement * Anemia * Bone lytic lesions/Back pain Multiple myeloma: Monoclonal M protein spike

What disease other than multiple myeloma has an M protein spike =IgM? How can it be differentiated?

Waldenstrom macroglobulinemia hyperviscosity syndrome (eg blurred vision, Raynaud phenomenon, no CRAB ssx)

monoclonal expansion of plasma cells but bone marrow shows \<10%

Monoclonal gammopathy of undetermined significance (MUGS) no CRAB ssx, can become multiple myeloma

Pseudo Pelger Huet anomaly is

a myelodysplastic syndrome neutrophils with bilobed nuclei typically seen after chemo

Kids with ALL and this mutation have a better prognosis

t(12:21)

smudge cells are seen in

CLL

What is Richter transformation?

CLL/SLL transformation into an aggressive lymphoma, most commonly DLBCL

dry tap on aspiration

hairy cell leukemia

hairy cell leukemia will stain positive for

TRAP - tartrate-resistant acid phosphatase has been replaced by flow cytometry for the most part

tx for hairy cell leukemia

cladribine, pentostatin

Aeur rods are found in

AML

myeloperoxidase positive cytoplasmic inclusions seen mostly in

APL (formerly M3 AML)

APL: t(15;17) responds to

all-trans retinoic acid (vit A), inducing differentiation of promyelocytes

Philadelphia chromosome t(9:22), BCR-ABL is in

CML

CML can accelerate and transform into

AML or ALL (blast crisis)

tx CML with

bcr-abl tyrosine kinase inhibitors like imatinib

what disorders are associated with V617F JAK2 mutation?

Chronic myeloproliferative disorders * polycythemia vera * essential thrombocytopenia * myelofibrosis

What is erythromelalgia and what does it occur in?

severe burning pain and red-blue coloration due to episodic blood clots in vessels of extremities polycythemia vera and essential thrombocythemia

Presents in a child as lytic bone lesions and skin rash or as recurrent otitis media with a mass involving the mastoid bone

langerhans cell histiocytosis

What are characteristics of cells in langerhans cell histiocytosis?

cells are functionally immature and do not effectively stimulate primary T cells via antigen presentation. Cells express S-100 (mesodermal origin) and CD1a Birbeck granules (tennis raquets or rod shaped on EM)

reverse dapigatran with what drug?

idarucizumab

What class of drugs do abciximab, eptifibatide, and tirofiban go into?

Gp IIb/IIIa inhibitors