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Path quick facts Flashcards

1
Q

Wire looping appearance on light microscopy of renal bx

A

Lupus nephritis

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2
Q

Crescent formation in the glomeruli

A

Rapid progressive glomerulonephritis

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3
Q

Anti-GBM abs, hematuria, hemoptysis

A

Goodpasture syndrome (rapid crescentic GN)

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4
Q

Deposits of IgA in the mesangium

A

Post-streptococcal glomerulonephritis (remember subepithelial humps)

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5
Q

Lumpy-bumpy deposits of IgG, IgM, and C3 in the mesangium

A

Acute post-streptococcal GN

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6
Q

Linear pattern of IgG deposition on immunofluorescence of kidney bx

A

Goodpasture syndrome (hemoptysis + hematuria)

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7
Q

Cola-colored urine + periorbital edema

A

Acute Post-strep GN

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8
Q

Crescents of fibrin and plasma proteins on bx + c-ANCA

A

Granulomatosis with polyangitis

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9
Q

Crescents of fibrin and plasma proteins on bx + p-ANCA

A

microscopic polyangitis

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10
Q

Renal pathology associated with Henoch-Schonlein purpura

A

IgA nephropathy (berger dz)

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11
Q

Eye problems (retinopathy, lens dislocation) + glomerulonephritis + sensorineural deafness

A

Alport syndrome (defect in Type IV collagen)

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12
Q

Nephritic syndrome and nephrotic syndrome, Tram-track appearance of GBM, subendothelial IC deposits, associated with hep B, hep C, lupus, subacute endocarditis

A

Membranoproliferative glomerulonephritis

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13
Q

Most common cause of nephrotic syndrome in kids, associated with recent infection, immunization or immune stimulus, tx with corticosteriods

A

minimal change disease

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14
Q

Associated with HIV, massive obesity, higher incidence in AA and Hispanics, effacement of foot processes

A

Focal segmental glomerulosclerosis

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15
Q

GBM thickening, “spike and dome” + subepithelial deposits, can be seen with SLE

A

Membranous nephropathy

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16
Q

deposits in mesangium, show apple-green birefringence with Congo red stain

A

Amyloidosis (seen in MM)

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17
Q

Eosinophilic nodular deposits = Kimmelstiel-Wilson lesions, mesangial expansion

A

Diabetic glomerulonephropathy (most common cause of end stage renal dz in US)

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18
Q

RBC casts

A

glomerulonephritis

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19
Q

WBC casts

A

acute pyelonephritis

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20
Q

Granular “muddy brown” casts

A

acute tubular necrosis

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21
Q

Waxy casts

A

end-stage renal disease

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22
Q

Radiopaque, envelope shaped kidney stone

A

Calcium oxalate

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23
Q

What are calcium oxalate stones associated with?

A

Chron’s dz (malabsorption), vitamin C abuse, ethylene glycol ingestion, vit C abuse, low citrate with low urine pH

-most common stone

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24
Q

Tx for calcium kidney stones

A

thiazides –> cause Ca2+ to be retained in serum

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25
Q

What infections are ammonium magnesium stones associated with?

A

Proteus mirabilis, Staph saprophyticus, Klebsiella

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26
Q

Radiopaque coffin lid shaped kidney stone

A

AMP (struvite)

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27
Q

Radioluscent rhomboid or rossette shaped kidney stone

A

uric acid stone

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28
Q

What are uric acid stones associated with?

A

hyperuricemia –> gout, Lesch-Nyhan

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29
Q

Radioluscent hexagonal kidney stone

A

cysteine stones

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30
Q

What are cysteine stones associated with?

A

Hereditary condition that prevents reabsorption of cysteine in the proximal tubule, begins in childhood

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31
Q

Most common childhood kidney tumor

A

Wilms tumor

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32
Q

What is the WAGR complex?

A

Wilms tumor, Aniridia (absent iris), Genitourinary malformations, Mental Retardation (deletion in WT1 gene)

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33
Q

Risk factors associated with transitional cell carcinoma

A

smoking, napthylamine dyes, aniline dyes, cyclophosphamide

most common tumor of urinary tract system

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34
Q

Risk factors for squamous cell carcinoma of the bladder

A

chronic cystitis, smoking, chronic nephrolithiasis, Schistosoma haematobium

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35
Q

Most common renal malignancy

A

Renal cell carcinoma - originates from PCT cells

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36
Q

Risk factors for renal cell carcinoma

A

smoking, men 50-70

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37
Q

What are the paraneoplastic syndromes associated with RCC?

A

2’ Polycythemia - excess EPO

secrete excess PTHrP –> bone resorption

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38
Q

Sources of ectopic EPO production and 2’ polycythemia

A

“Potentially Really High Hematocrit” - Pheocromocytoma, Renal cell carcinoma, Hepatocellular carcinoma, Hemangioblastoma

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39
Q

Causes of polycythemia

A

Hypoxia - lung dz, high altitude
Ectopic EPO
Primary Polycythemia
Down syndrome

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40
Q

Blistering cutaneous photosensitivity, tea colored urine, hypertrichosis, facial hyperpigmentation, can be associated with alcohol or Hep C (increased LFTs)

A

Porphyria cutanea tarda

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41
Q

Defective enzyme in porphyria cutanea tarda

A

Uroporphyrinogen decarboxylase - converts uroporphyrinogen III to coprophyrinogen

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42
Q

Painful abdomen, port-wine colored urine, polyneuropathy, psychological disturbances, precipitated by drugs

A

Acute intermittent porphyria

Tx = heme and glucose to inhibit ALA synthase

43
Q

Defective enzyme in Acute intermittent porphyria

A

Porphobilinogen deaminase - converts porphobilinigen to hydroxymethylbilane

44
Q

Rate limiting step in heme synthesis

A

Delta-aminolevulinic acid synthase (ALA synthase) - requires B6 cofactor

45
Q

Enzymes inhibited by lead in the heme synthesis pathway

A

Ferrochelatase, Delta-aminolevulinic acid dehydratase

46
Q

Blue lines on gum, hyperluscent lines on long bones in X-ray, abdominal pain, growth impairment, HA, hearing problems, wrist/foot drop, encephalopathy, basophilic stippling

A

Lead poisoning

Tx - succimer + dimercaprol in kids, EDTA in adults

47
Q

Acanthocyte (spur cell)

A

Abetalipoproteinemia, liver dz

48
Q

Basophilic stippling

A

lead poisoning

49
Q

Teardrop cell (dacrocyte)

A

Bone marrow infiltration

50
Q

Bite cell (degmacyte)

A

G6PD def.

51
Q

Echinocyte (burr cell)

A

Liver dz, renal dz, pyruvate kinase def

52
Q

Elliptocyte

A

hereditary elliptocytosis

53
Q

Ringed sideroblast

A

disorders of heme synthesis - found on BM bx

54
Q

Schistocytes

A

DIC, TPP/HUS, HELLP syndrome - fragments of RBCs

55
Q

Spherocyte

A

Hereditary spherocytosis

56
Q

Target cells

A

“HALT at target” - HbC dz, Asplenia, Liver dz, Thalassemia

57
Q

Heinz bodies

A

G6PD

caused by oxidized Hb molecules that precipitate, can become bite cells

58
Q

Howell-Jolly bodies

A

Asplenia

-nuclear remnants found in RBCs, only 1/cell

59
Q

Causes of hypochromic, microcytic anemia

A

iron deficiency, lead poisoning, thalassemia

60
Q

Skull x-ray shows hair-on-end appearance

A

Beta thalassemia, sickle cell disease

61
Q

decreased serum iron, increased TIBC/transferrin, decreased ferritin, decreased transferrin saturation

A

IDA

62
Q

decreased serum iron, decreased TIBC/transferrin, increased ferritin (high stores)

A

AOCD

63
Q

increased serum iron, increased ferritin, increased transferrin saturation

A

Hemochromatosis

64
Q

Causes of microcytic anemia

A

Iron deficiency, late anemia of chronic dz, thalassemias, lead poisoning, sideroblastic anemia

65
Q

Alpha-globin gene deletions, Asian and African populations, form excess beta globin chains and form HbH (four beta globin chains pair)

A

Alpha thalassemia

66
Q

Beta-globin absent or underproduced, increase in HbF, crew cut look on x-ray due to marrow expansion, target cells, Mediterranean populations, chipmunk face

A

Beta thalassemia - tx with blood transfusions

minor = increased HbA2

67
Q

What is the genetic cause of sideroblastic anemia?

A

defect in delta-ALA synthase gene (also caused by drugs, alcohol, B6 def., isoniazid)

tx - B6 supplement (cofactor for ALA synthase)

68
Q

Microcytic anemia + swallowing difficulty + glossitis

A

Plummer-Vinson syndrome (iron deficiency with esophageal webs)

69
Q

Causes of megaloblastic anemia (hypersegmented neutrophils)

A

Folate deficiency, B12 deficiency, orotic aciduria

-can be decreased intake, increased requirement (preggers), mtx or TMP-SMX

70
Q

Neuro sx, increased homocysteine, neuro sx, megaloblastic anemia, increased methylmalonic acid, hypersegmented neutrophils

A

B12 deficiency

-can be seen in strick vegans, malabsorption, Diphyllobothrium latum

71
Q

Increased homocysteine, megaloblastic anemia, normal methylmalonic acid, hypersegmented neutrophils

A

Folate deficiency

72
Q

Causes of non-megaloblastic macrocystic anemia

A

Liver disease, alcoholism

73
Q

Mechanism of AOCD

A

inflammation –> increases hepcidin which binds all ferroportin in macs and intestinal cells –> can’t transport iron to bone marrow for RBC synthesis

74
Q

Drugs that cause aplastic anemia

A

Chloramphenicol, Cancer drugs, Benzene, Zidovudine (HIV tx)

75
Q

Causes of aplastic anemia

A 
Radiation 
Fanconi anemia 
Drugs 
Infections - EBV, Parvo B19, HIV, hepatitis 
Idiopathic
76
Q

Pancytopenia + hypocellular bone marrow with fatty infiltrate

A

Aplastic anemia

-can tx with GM-CSF

77
Q

Elevated creatine + normocytic anemia

A

renal failure (can’t make EPO)

78
Q

Spherocytes on smear, splenomegaly, increased MCHC, increased RDW, + osmotic fragility test

A

Hereditary spherocytosis

-tx = splenomegaly

79
Q

Hemolytic anemia in a newborn

A

think about pyruvate dehydrogenase def.

80
Q

Glutamic acid –> lysine mutation in Beta-globin gene

A

HbC disease - milder than sickle cell dz

81
Q

point mutation in beta-globin gene glutamic acid –> valine

A

Sickle cell anemia

82
Q

Precipitating factors for HbS to polymerize in sickle cell anemia

A

low O2, high altitude, dehydration, acidosis

83
Q

Treatment for sickle cell anemia

A

Hydroxyurea - increases concentration of HbF

84
Q

Complications of sickle cell anemia

A

aplastic crisis with ParvoB19, autosplenectomy (vax for S. pneumo and Hib), splenic infarcts, Salmonella osteomyelitis, painful crisis in hands and feet or acute chest syndrome (vaso-occlusion), Renal papillary necrosis

85
Q

Causes of Warm AI hemolytic anemia

A

congenital immune issues, CLL, EBV, HIV, SLE

-IgG ab

86
Q

Causes of Cold AI hemolytic anemia

A

EBV mononucleosis, Mycoplasma pneumonia, EBV, CLL

-IgM ab + complement destruction

87
Q

Test for AI hemolytic anemia

A

Coombs

  • direct = testing if RBCs are coated with Ig
  • indirect = testing for presence of anti-RBC Ig in serum
88
Q

Painful cyanosis in fingers and toes with hemolytic anemia

A

Cold AI hemolytic anemia

89
Q

Red urine in the morning and fragile RBCs, hemosiderinuria thrombosis

A

Paroxysmal nocturnal hemoglobinuria

90
Q

Labs seen with von Willebrand disease

A

increased bleeding time and PTT

91
Q

What is the defect in von Willebrand disease?

A

defect in vWF which normally protects Factor VIII (intrinsic pathway) –> increase PTT
defect in vWF which helps platelets form plug –> increase bleeding time

92
Q

most common inherited bleeding disorder

A

von Willebrand disease

93
Q

Lab used to diagnose von Willebrand disease

A

ristocetin cofactor assay - won’t see platelet aggregation

94
Q

Treatment for von Willebrand disease

A

desmopressin - causes vWF to be released

95
Q

Labs seen with DIC

A

schistocytes, increase in D-dimer, decrease in fibrinogen,

increase in BT, PT, PTT, decreased platelets

96
Q

Causes of DIC

A

“STOP Making Thrombin” - Sepsis (gram -), Trauma, Obstetric complications (placental abruption, amniotic fluid embolism), Pancreatitis (acute), Malignancy, Transfusions

97
Q

Clinical signs of a platelet disorder

A

microhemorrhages - petechiae, purpura, epistaxis, mucous membrane bleeding
increased bleeding time

98
Q

Symptoms of TTP

A

“FAT RN” - Fever, Anemia (microangiopathic hemolytic), Thrombocytopenia, Renal Failure, Neuro symptoms

99
Q

Defect in Thrombotic thrombocytopenic purpura

A

inhibition of deficiency in ADAMTS 13 - can’t break down large vWF multimers –> they bind platelets –> platelets aggregate and thrombosis
-generally an acute disease

100
Q

Defect in Immune Thrombocytopenia

A

anti-GpIIb/IIIa antibodies –> splenic macs consume platelet/ab complexes –> decreased platelets

  • often follows viral illness
  • see megakaryocytes on BM bx
  • tx with steroids or IVIG
101
Q

Defect in Glanzman thrombocytopenia

A

defect in GpIIb/IIIa –> can’t form platelet-platelet aggregation
-normal platelet count, just increased BT

102
Q

Defect in Bernard-Soulier syndrome

A

defective GpIb –> platelets can’t bind vWF

-normal platelet count, increased BT

103
Q

Triad of HUS

A

thrombocytopenia, renal failure, microangiopathic hemolytic anemia
-milder form of TTP

104
Q

What is a common illness that affects children before they have HUS?

A

E. coli O157:H7 infection causes diarrhea - produces shiga toxin

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