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Hematology/Oncology > Pathology > Flashcards

Pathology Flashcards

1
Q

Normocytic anemias

A

Nonhemolytic (retic count normal or decreased) - ACD (inflammation –> hepcidin from liver, which binds ferroportin on int and macrophages, inhibiting Fe transport) and Fe deficiency (early), aplastic anemia (failure/destruction of myeloid stem cells), CKD

Hemolytic (retic count elevated)

  • Intrinsic: RBC membrane defect (hereditary spherocytosis), RBC enzyme deficiency (G6PD, pyruvate kinase), HbC defect, PNH, Sickle cell
  • Extrinsic (autoimmune, angiopathic [micro or macro], infxn)
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2
Q

Macrocytic anemias

A

MCV > 100fL

Megaloblastic (folate or B12 deficiency, orotic aciduria)

Non-megaloblastic (liver dz, alcohol abuse, reticulocytosis)

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3
Q

Symptoms of iron def anemia

A

Fatigue, conjunctival pallor, spoon nails (koilonychia) - due to decreased iron from bleeding, malnutrition/abs or increased demand (eg preg) –> decreased heme synth and microcytosis + hypochromia

May manifest as Plummer-Vinson

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4
Q

Microcytic anemias

A 
Iron deficiency (late)
ACD (late)
Thalassemias
Lead poisoning
Sideroblastic anemia
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5
Q

Alpha thalassemia

A

Alpha globin gene deletion –> less alpha globin synthesis

if 4 alleles are deleted, get excess gamma globin –> Hb Barts (hydrops fetalis)

if 3 alleles are deleted, get excess beta globin –> HbH disease

if 1-2 alleles are deleted, get less severe anemia

Causes microcytic anemia

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6
Q

Beta thalassemia

A

Point mutation in splice sites and promoter sequences –> decreased beta globin sequence

Heterozygote = beta thal minor (some beta chain, increased HbA2, usu asymp)

Homozygote (beta thal major) - no beta globin –> severe microcytic anemia (anisocytosis, poikilocytosis, target cells, schistocytes), marrow expansion –> skel deformities (crew cut on Xray, chipmunk facies), + extramedullary hematopoiesis (hepatosplenomeg); baby has lots of HbF so can get by without sx for first 6 months

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7
Q

Lead poisoning - pathophys + sx

A

Pathophys: decreased heme synth and increased RBC protoporphyrin + inhibition of rRNA degrad –> basophilic stippling

Sx: lead (“burton”) lines on gingivae + metaphyses, encephalopathy, stippling of RBCs, abdominal colic, sideroblastic anemia, wrist and foot drop

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8
Q

Lead poisoning tx

A

EDTA and dimercaprol

Kids: succimer (chelation)

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9
Q

Burton lines

A

Lines on gingivae in lead poisoning

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10
Q

Treatment of sideroblastic anemia

A

Pyridoxine (vitamin B6) - cofactor for ALA synthase, which catalyzes the RLS of heme synth

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11
Q

Megaloblastic anemia findings

A

Macrocytosis, hypersegmented PMNs, glossitis

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12
Q

Folate deficiency findings and sx

A

Increased homocysteine, normal MMA

no neuro sx

macrocytic anemia

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13
Q

B12 deficiency - findings + sx

A

Findings: increased homocysteine and MMA

neuro sx

macrocytic anemia

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14
Q

Orotic aciduria

A

Defect in UMP synthase so can’t turn orotic acid into UMP in de novo pyrimidine synth

sx: children - failure to thrive, dev delay, megaloblastic (macrocytic) anemia that doesn’t get better with folate and b12
tx: give UMP to bypass bad enzyme

NO HYPERAMMONEMIA (unlike ornithine transcarbamylase deficiency)

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15
Q

Nonmegaloblastic macrocytic anemia

A

DNA synthesis isn’t impaired, so get macrocytosis without hypersegmented polys

causes: alcohol, liver dz, hypothyroid, reticulocytosis

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16
Q

Alpha thalassemia

A

Alpha globin gene deletion –> less alpha globin synthesis

if 4 alleles are deleted, get excess gamma globin –> Hb Barts (hydrops fetalis)

if 3 alleles are deleted, get excess beta globin –> HbH disease

if 1-2 alleles are deleted, get less severe anemia

Causes microcytic anemia

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17
Q

Beta thalassemia

A

Point mutation in splice sites and promoter sequences –> decreased beta globin sequence

Heterozygote = beta thal minor (some beta chain, increased HbA2, usu asymp)

Homozygote (beta thal major) - no beta globin –> severe microcytic anemia (anisocytosis, poikilocytosis, target cells, schistocytes), marrow expansion –> skel deformities (crew cut on Xray, chipmunk facies), + extramedullary hematopoiesis (hepatosplenomeg); baby has lots of HbF so can get by without sx for first 6 months

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18
Q

Lead poisoning - pathophys + sx

A

Pathophys: decreased heme synth and increased RBC protoporphyrin + inhibition of rRNA degrad –> basophilic stippling

Sx: lead (“burton”) lines on gingivae + metaphyses, encephalopathy, stippling of RBCs, abdominal colic, sideroblastic anemia, wrist and foot drop

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19
Q

Lead poisoning tx

A

EDTA and dimercaprol

Kids: succimer (chelation)

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20
Q

Burton lines

A

Lines on gingivae in lead poisoning

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21
Q

Treatment of sideroblastic anemia

A

Pyridoxine (vitamin B6) - cofactor for ALA synthase, which catalyzes the RLS of heme synth

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22
Q

Megaloblastic anemia findings

A

Macrocytosis, hypersegmented PMNs, glossitis

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23
Q

Folate deficiency findings and sx

A

Increased homocysteine, normal MMA

no neuro sx

macrocytic anemia

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24
Q

B12 deficiency - findings + sx

A

Findings: increased homocysteine and MMA

neuro sx

macrocytic anemia

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25
Q

Orotic aciduria

A

Defect in UMP synthase so can’t turn orotic acid into UMP in de novo pyrimidine synth

sx: children - failure to thrive, dev delay, megaloblastic (macrocytic) anemia that doesn’t get better with folate and b12
tx: give UMP to bypass bad enzyme

NO HYPERAMMONEMIA (unlike ornithine transcarbamylase deficiency)

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26
Q

Nonmegaloblastic macrocytic anemia

A

DNA synthesis isn’t impaired, so get macrocytosis without hypersegmented polys

causes: alcohol, liver dz, hypothyroid, reticulocytosis

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27
Q

Signs of intravascular hemolysis

A

Decreased haptoglobin

Increased LDH

Schistocytes and increased retics on smear

Hemoglobinuria, hemosiderinuria, urobilinogen in the urine

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28
Q

How to distinguish intravasc from extravasc hemolysis

A

Extravasc - no hemoglobin or hemosiderin in urine

Spherocytes on peripheral smere

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29
Q

Hereditary spherocytosis

A

Intrinsic hemolytic normocytic anemia

Defect in proteins in RBC mem and PM skeleton –> small, round RBCs that are cleared by spleen (decreased SA:volume ratio, so increased MCHC)

Diag via osmotic fragility test

Tx: splenectomy

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30
Q

G6PD deficiency

A

X-linked mutation –> decreased glutathione so increased RBC susc to oxidant stress

31
Q

Pyruvate kinase deficiency

A

Aut recessive

Intrinsic hemolytic normocytic anemia

Mutation –> decreased ATP –> rigid RBCs

32
Q

Paroxysmal nocturnal hemoglobinuria

A

Acquired mutation in hemat. stem cell –> decreased synth of GPI anchor for decay-accelerating factor (CD55), which protects RBC membrane from complement –> increased complement-mediated RBC lysis

Coombs -, pancytopenia, venous thrombosis

tx: eculizumab (complement inhib)

33
Q

Pathophys of sickle cell disease

A

Point mut –> AA replacement in beta chain of hemoglobin, so at low O2, high altitude or when acidotic, deoxygenated HbS polymerizes –> anemia and vaso-occlusive crises

34
Q

Complications of sickle cell

A

Aplastic crisis (with parvo)

Autosplenectomy (will see Howell-Jolly bodies)

Splenic infarct/sequestration

Salmonella osteomyelitis

Painful crises due to vaso-occlusion, avasc necrosis etc

Renal papillary necrosis (decreased O2 to kidneys) and microhematuria (from medullary infarct)

35
Q

Sickle cell tx

A

hydroxyurea to increase HbF + hydration

36
Q

AIHA - two types

A

Warm agglutinin (IgG - chronic anemia seen in SLE, CLL, and with drugs)

Cold agglutinin IgM - set off by cold and seen in CLL, mycoplasma pneumo, mono)

Usually coombs +

37
Q

Primary iron storage protein of body

A

Ferritin

38
Q

Leukopenias - cell counts

A

Neutropenia and lymph opens (ANC

39
Q

Effect of corticosteroids on leukocyte numbers

A

Increased PMNs but decreased activation of adhesion mcs so they can’t migrate to sites of inflamm

Sequestration of eos in ln (eosinopenia)

Apop of lymphocytes (lymphopenia)

40
Q

Porphyrias

A
  1. Acute intermittent porphyria - dysfxal porphobilinogen deaminase –> buildup of heme precursors –> 5P’s:
Painful abdomen
Port-wine-colored urine
Polyneuropathy
Psych disturbances
Precipitated by drugs (eg P450 inducers), alc, starvation

Tx: glucose and heme (inhibit ALA synthase, so don’t get buildup of delta-ALA)

  1. Porphyria cutanea tarda - defective uroporphyrinogen decarboxylase –> buildup of uroporphyrin, which –> tea-colored urine, blistering cut lesions that = photosensitive
41
Q

PT vs PTT

A

PT tests common and extrinsic pathways (factors I, II, V, VII, X)

PTT tests common and intrinsic pathways (all factors except 7 and 8)

42
Q

Coagulation disorders

A

Hemophilia (intrinsic pathway):
A = def factor 8 (X rec)
B = def factor 9 (X rec)
C = def factor 11 (aut rec)

hemarthroses, easy bruising etc
tx = desmopressin + factor that’s missing

43
Q

Platelet disorders

A

Bernard-Soulier (Decreased GPIb –> no plt-to-vWF binding)

Glanzmann thrombasthenia (Decreased GPIIb/IIIa –> no plt-fibrin-plt adhesion)

Immune thrombocytopenia (anti-GPIIb/IIIa antibodies –> plt-Ig complex is cleared in spleen)

TTP (inhib or deficiency of ADAMTS13 –> vWF doesn’t get cut and long multimers increase plt adhesion –> agg and thrombosis)

All of these decrease plt count and increase bleeding time

44
Q

von WIllebrand disease

A

Decreased vWF –> decreased factor 8 (intrinsic pathway) and decreased plt-to-vWF adhesion

aut dom

tx: desmopressin to release vWF stored in endothelium

45
Q

DIC

A

Widespread activation of clotting –> deficiency of clotting factors –> bleeding

Causes: (STOP Making New Thrombi)
Sepsis
Trauma
Ob complications
Pancreatitis
Malig
Nephrotic Syndrome
Transfusion
46
Q

Causes of hypercoag

A

Antithrombin deficiency (inherited or acquired due to renal failure/nephrotic syndrome – lose antithrombin in urine)

Factor V Leiden (mutant factor V isn’t degraded by protein C –> keep clotting)

Protein C or S deficiency (can’t inactivatey factors 5a and 8a) - DON’T GIVE WARFARIN (can –> thrombotic skin necrosis)

Prothrombin gene mut –> increased prothrombin

47
Q

Types of blood transfusion:

A

Packed RBCs (acute blood loss or severe anemia)

Plts (to stop significant bleeding)

Fresh frozen plasma (to increase coag factors in context of DIC, cirrhosis, immediate warfarin reversal)

Cryoprecipitate (to increase fibrinogen, factor 8, factor 13)

48
Q

Leukemoid rxn

A

Acute inflamm response to infxn

Increased white count with increased leukocyte alk phos (unlike in CML)

49
Q

Leukemia vs. lymphoma

A

Leukemia - widespread in marrow (unreg growth and diff of WBCs in marrow –> marrow failure), tumor cells in periph blood

Lymphoma - discrete tumor mass in ln

50
Q

Hodgkin vs. non-hodgkin lymphoma

A

Hodgkin - localized tumor with contiguous spread, extranodal involvement = rare; Reed-Sternberg cells + EBV assoc

non-hodgkin: multiple, periph nodes + extranodal involvement, noncontig spread - much worse; assoc with HIV and autoimmune dz and usu involve B cells

51
Q

Reed Sternberg cells - pathognomonic for what disease? Which CDs? Prognostic factors?

A

Hodgkin lymphoma

CD 15 and 30

Better prog if strong stromal or lymphocytic reaction against them

“Owl eye” tumor giant cells - binucleate or bilobed

52
Q

“Starry sky” histo

A

lots of lymphocytes (small, dark cells) with macrophages interspersed

Characteristic of Burkitt lymphoma

53
Q

Burkitt lymphoma genetics

A

t(8;14) translocation of c-myc from chrom 8 and heavy-chain Ig from chrom 14

54
Q

Diffuse large B-cell lymphoma

A

non-hodgkin lymphoma

most common one in adults

55
Q

Follicular lymphoma genetics

A

t(14;18) - BCL2 on chrom 18 and heavy-chain Ig on chrom 14

Indolent, usu adults
non-hodgkin

56
Q

Mantle-cell lymphoma genetics

A

non-hodgkin, older males

t(11;14) - cyclin D1 on chrom 11, heavy-chain Ig on chrom 14

CD5+

57
Q

Cause and sx of adult t-cell lymphoma

A

Cause = HTLV (assoc with IVDU)

Cutaneous lesions + lytic bone lesions (hyperCa)

58
Q

Mycosis fungoides

A

Adult t cell lymphoma, with cutaneous lesions + atypical CD4 cells with “cerebriform” nuclei

May progress to Sezary syndrome

59
Q

Sezary syndrome

A

T-cell leukemia that may develop from mycosis fungoides

60
Q

Sx of multiple myeloma

A

CRAB

HyperCalcemia
Renal failure
Anemia
Bone lytic lesions

Also assoc with increased susc to infxn, primary amyloidosis

61
Q

Lab findings with multiple myeloma

A

M spike on serum protein electrophoresis from excess Ig (G or M)

Ig light chains in urine (Bence Jones protein)

Rouleaux formation of RBCs on smear

Plasma cells with “clock-face” chromatin and intracytoplasmic inclusions with Ig on histo

62
Q

Bence Jones protein

A

Ig light chains in urine, seen in multiple myeloma

63
Q

Monoclonal gammopathy of undetermined significance vs. Multiple Myeloma vs. Waldenstrom macroglobulinemai

A

MGUS - monoclonal expansion of plasma cells is asymptomatic, but may –> MM

Waldenstrom - increased IgM –> hyperviscosity of blood, which can blur vision, cause Raynaud’s etc, but no CRAB sx

64
Q

Myelodysplastic syndromes

A

Stem cell problem –> ineffective hematopoiesis of non-lymphocytes

Can be de novo mut or environmental (eg chemo)

May –> AML

Ex: Pseudo-pelger huet syndrome (PMNs are only bilobed)

65
Q

Leukemias

A

Lymphoid:
ALL
Small lymphocytic lymphoma/chronic lymphocytic leukemia
Hairy cell leukemia

Myeloid:
Acute myelogenous leukemia
Chronic myelog. leukemia

66
Q

Acute lymphoblastic leukemia/lymphoma

A

Kids

Can be pre-T (TdT+) or pre-B (TdT and CD10+) cells (T often presents as mediastinal mass)

See increased lymphoblasts in periph blood and marrow

t(12;21) = better prog

responsive to therapy

67
Q

Small lymphocytic lymphoma/chronic lymphocytic leukemia

A

> 60yo, most common adult leukemia

B cell neoplasm, so CD20 and CD5+

progresses slowly, asymp

smudge cells on smear

CLL = SLL but with increased periph blood and marrow lymphocytosis

68
Q

Hairy cell leukemia

A

Elderly

Mature B cell tumor

marrow fibrosis and filamentous projections on cells

tx: cladribine, pentostatin

69
Q

Acute myelogenous leukemia

A

~65yo

Lab findings: Auer rods, peroxidase positive cyt inclusions, lots of myeloblasts on smear

assoc with Down syndrome; also t(15;17), alkylating chemo and radn, myeloproliferative disorders

May present with DIC

70
Q

Chronic myelogenous leukemia

A

~64yo

Philadelphia chrom!! t(9;22) - BCR-ABL –> myeloid stem cell prolif, may –> blast crisis (AML or ALL)

tx: imatinib (inhibit bcr-abl tyrosine kinase)

71
Q

Langerhans cell histiocytosis

A

Proliferative disorders of dendritic cells

sx: lytic bone lesions, skin rash, recurrent otitis media (kids) with mass on mastoid bone

cells = immature and don’t effectively present antigen to T ccells

cells express S-100 (mesoderm) and CD1a (MHC) + have Birbeck granules (tennis rackets)

72
Q

Mutation associated with many chronic myeloproliferative disorders (except CML)

A

JAK2

73
Q

Polycythemia vera

A

Disorder of increased hematocrit, usu assoc with JAK2 mut

sx: erythromelalgia (burning pain and red-blue discoloration due to episodic blood clots in extremities) + burning with hot shower (increased basophils)

Hematology/Oncology (4 decks)

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