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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
Orotic aciduria
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)
26
Nonmegaloblastic macrocytic anemia
DNA synthesis isn't impaired, so get macrocytosis without hypersegmented polys causes: alcohol, liver dz, hypothyroid, reticulocytosis
27
Signs of intravascular hemolysis
Decreased haptoglobin Increased LDH Schistocytes and increased retics on smear Hemoglobinuria, hemosiderinuria, urobilinogen in the urine
28
How to distinguish intravasc from extravasc hemolysis
Extravasc - no hemoglobin or hemosiderin in urine Spherocytes on peripheral smere
29
Hereditary spherocytosis
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
30
G6PD deficiency
X-linked mutation --> decreased glutathione so increased RBC susc to oxidant stress
31
Pyruvate kinase deficiency
Aut recessive Intrinsic hemolytic normocytic anemia Mutation --> decreased ATP --> rigid RBCs
32
Paroxysmal nocturnal hemoglobinuria
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
Pathophys of sickle cell disease
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
Complications of sickle cell
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
Sickle cell tx
hydroxyurea to increase HbF + hydration
36
AIHA - two types
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
Primary iron storage protein of body
Ferritin
38
Leukopenias - cell counts
Neutropenia and lymph opens (ANC
39
Effect of corticosteroids on leukocyte numbers
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
Porphyrias
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) 2. Porphyria cutanea tarda - defective uroporphyrinogen decarboxylase --> buildup of uroporphyrin, which --> tea-colored urine, blistering cut lesions that = photosensitive
41
PT vs PTT
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
Coagulation disorders
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
Platelet disorders
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
von WIllebrand disease
Decreased vWF --> decreased factor 8 (intrinsic pathway) and decreased plt-to-vWF adhesion aut dom tx: desmopressin to release vWF stored in endothelium
45
DIC
Widespread activation of clotting --> deficiency of clotting factors --> bleeding ``` Causes: (STOP Making New Thrombi) Sepsis Trauma Ob complications Pancreatitis Malig Nephrotic Syndrome Transfusion ```
46
Causes of hypercoag
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
Types of blood transfusion:
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
Leukemoid rxn
Acute inflamm response to infxn Increased white count with increased leukocyte alk phos (unlike in CML)
49
Leukemia vs. lymphoma
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
Hodgkin vs. non-hodgkin lymphoma
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
Reed Sternberg cells - pathognomonic for what disease? Which CDs? Prognostic factors?
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
"Starry sky" histo
lots of lymphocytes (small, dark cells) with macrophages interspersed Characteristic of Burkitt lymphoma
53
Burkitt lymphoma genetics
t(8;14) translocation of c-myc from chrom 8 and heavy-chain Ig from chrom 14
54
Diffuse large B-cell lymphoma
non-hodgkin lymphoma most common one in adults
55
Follicular lymphoma genetics
t(14;18) - BCL2 on chrom 18 and heavy-chain Ig on chrom 14 Indolent, usu adults non-hodgkin
56
Mantle-cell lymphoma genetics
non-hodgkin, older males t(11;14) - cyclin D1 on chrom 11, heavy-chain Ig on chrom 14 CD5+
57
Cause and sx of adult t-cell lymphoma
Cause = HTLV (assoc with IVDU) Cutaneous lesions + lytic bone lesions (hyperCa)
58
Mycosis fungoides
Adult t cell lymphoma, with cutaneous lesions + atypical CD4 cells with "cerebriform" nuclei May progress to Sezary syndrome
59
Sezary syndrome
T-cell leukemia that may develop from mycosis fungoides
60
Sx of multiple myeloma
CRAB HyperCalcemia Renal failure Anemia Bone lytic lesions Also assoc with increased susc to infxn, primary amyloidosis
61
Lab findings with multiple myeloma
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
Bence Jones protein
Ig light chains in urine, seen in multiple myeloma
63
Monoclonal gammopathy of undetermined significance vs. Multiple Myeloma vs. Waldenstrom macroglobulinemai
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
Myelodysplastic syndromes
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
Leukemias
Lymphoid: ALL Small lymphocytic lymphoma/chronic lymphocytic leukemia Hairy cell leukemia Myeloid: Acute myelogenous leukemia Chronic myelog. leukemia
66
Acute lymphoblastic leukemia/lymphoma
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
Small lymphocytic lymphoma/chronic lymphocytic leukemia
>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
Hairy cell leukemia
Elderly Mature B cell tumor marrow fibrosis and filamentous projections on cells tx: cladribine, pentostatin
69
Acute myelogenous leukemia
~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
Chronic myelogenous leukemia
~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
Langerhans cell histiocytosis
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
Mutation associated with many chronic myeloproliferative disorders (except CML)
JAK2
73
Polycythemia vera
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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