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Heme-Onc > Pathology > Flashcards

Pathology Flashcards

1
Q

Microcytic anemias

A

Iron deficiency, anemia of chronic disease, thalassemias, lead poisoning, sideroblastic anemia

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2
Q

Normocytic anemias

A

Hemolytic or non-hemolytic (chronic disease, aplastic anemia)

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3
Q

Macrocytic anemias

A

Megaloblastic (folate/B12 defciciency), non-megaloblastic (liver disease, alcoholism)

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4
Q

Iron deficiency anemia

A

MICROCYTIC - chronic bleeding, malnutrition, or increased demand – decreased iron and ferritin, increased TIBC (transferrin) – fatigue, conjunctival pallor, spoon nails, difficulty swallowing

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5
Q

Plummer Vinson syndrome

A

Plumbers DIE – Dysphagia, Iron defciency anemia, Esophageal webs

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6
Q

a-thalassemia

A

MICROCYTIC - a-globin gene deletions (chromosome 16) – cis (Asians) or trans (African) – 4 deletions (Hb barts - y4 - hydrops fetalis), 3 deletions (HbH disease)

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7
Q

B-thalassemia

A

MICROCYTIC - point mutations in Kozak sequence (chromosome 11) - Mediterranean – defective mRNA processing – minor (heterozygote) - Less B chain, asymptomatic, increased HbA2 – major (homozygote) - no B chain (have HbF), requires blood transfusions – crew cut on x-ray, skeletal deformities, chipmunk facies, extramedullary hematopoiesis (increased risk of parvovirus B12 aplastic crisis)

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8
Q

Lead poisoning

A

MICROCYTIC – Inhibits ferrochelatase and ALA dehydratase – increase RBC protoporhyrin IX and d-ALA and decrease heme – basophilic stippling (retained aggregates of rRNA) – LEAD (Lead Lines on gingiva and bones, Encephalopathy, Abdominal colic and sideroblastic Anemia, Drops (wrist and foot) – Tx: Dimercaprol and EDTA (Succimer for kids)

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9
Q

Sideroblastic anemia

A

Defect in heme synthesis — X-linked defect in d-ALA synthase – can be acquired, alcohol, lead, vitamin B6 deficiency, copper deficiency, isoniazid) – ringed sideroblasts (iron-laden PRUSSIAN BLUE STAIN mitochondria) – increased iron/ferritin, normal/decreased TIBC – Tx: Pyridoxine (B6)

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10
Q

Hepcidin

A

Made in liver (decreases intestinal iron absorption and iron release from macrophages) – increased when iron is low

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11
Q

Pure RBC Aplasia

A

Microcytic, hypochromic – CD8/IgG attack RBC precursors — d/t parvovirus B19, thymoma, leukemia

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12
Q

Folate deficiency

A

MEGALOBLASTIC (hypersegmented PMNs) – impairs purine/pyrimidine synthesis – d/t malnutrition, malabsorption (jejunum), drugs (methotrexate, TMP, phenytoin), pregnancy – INCREASE HOMOCYSTEINE, NORMAL METHYLMALONIC ACID – No neurologic symptoms

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13
Q

B12 (cobalamin) deficiency

A

MEGALOBLASTIC – Insufficient intake, malabsorption (ileum), pernicious anemia (no intrinsic factor), gastrectomy – INCREASED HOMOCYSTEINE AND METHYLMALONIC ACID – Neurologic symptoms (degeneration of spinocerebellar tract, lateral corticospinal tract, and dorsal column

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14
Q

Orotic aciduria

A

Can’t convert orotic acid to UMP (defective UMP syntahse) – Failure to thrive, developmental delay, megaloblastic anemia refractory to folate/B12 – Orotic acid in urine – Tx: Uridine monophosphate

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15
Q

Schilling Test

A

Give oral B12 – if normal urinary excretion then dietary deficiency — if decreased excretion then impaired absorption (can further test with intrinsic factor)

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16
Q

Intravascular hemolysis

A

Decreased haptoglobin, increased LDH, schistocytes, increased reticulocytes — hemoglobin, hemosiderin, and urobilinogen in urine

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17
Q

Extravascular hemolysis

A

Macrophages in spleen destroy RBCs – spherocytes, increased LDH, increased unconjugated bilirubin

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18
Q

Anemia of chronic disease

A

Increased hepcidin, decreased iron, decreased TIBC, increased ferritin (storage) – Tx: EPO for chronic kidney disease

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19
Q

Aplastic anemia

A

Myeloid cell issue due to radiation and drugs (chloramphenicol), viral agents (B19, EBV, HIV, HCV), Fanconi anemia, or idiopathic – pancytopenia (increased EPO due to compensation), hypocellular bone marrow with fat infiltrate – fatigue, pallor, mucosal bleeding, petechiae, infection – Tx: immunosuppression, bone marrow allograft, RBC/platelet transfusion, bone marrow stimulants

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20
Q

Hereditary spherocytosis (E)

A

Defect in ankyrin, band 3, protein 4.2, spectrin (membrane proteins) — increased MHC, decreased haptoglobin, increased LDH, increased reticulocytes — splenomegaly, aplastic crisis, risk for gallstones – positive osmotic fragility test – Tx: splenectomy

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21
Q

G6PD deficiency (I/E)

A

X-linked recessive — abnormal hexose monophosphate shunt (decreased glutathione so increased oxidative damage) — anemia following oxidative stress (sulfa drugs, dapsone, antimalarials, infections, fava beans) — Heinz bodies, bite cells

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22
Q

Pyruvate kinase deficiency (E)

A

Hemolytic anemia in a newborn - autosomal recessive – decreased ATP

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23
Q

HbC defect (E)

A

Glutamic acid to lysine mutation in B-globin

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24
Q

Paroxysmal nocturnal hemoglobinuria (I)

A

Increased complement mediated RBC lysis – increased acute leukemias — pancytopenia, aplastic anemia, Coombs negative, venous thrombosis — CD55/59 negative — Tx: Eculizumab

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25
Q

Sickle cell anemia (E)

A

Glutamic acid to valine substitution allows hydrophobic interaction between Hb in anoxic conditions — low O2, high altitude, or acidosis leads to anemia and vaso-occlusive disease — newborns are asymptomatic because HbF is protective – heterozygotes have malaria resistance — Crew cut on skull x-ray (marrow expansion) – decreased haptoglobin — Tx: hydroxyurea (increases HbF)

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26
Q

Sickle cell complications

A

Aplastic crisis (parvovirus B19) – autosplenectomy (Howell-Jolly bodies, increased risk of encapsulated organisms) – splenic infarct (microvascular infarcts) – Salmonella osteomyelitis – painful crises (vaso-occlusive - acute chest syndrome, avascular necrosis, stroke) – renal papillary necrosis

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27
Q

Autoimmune hemolytic anemia

A

Warm agglutinin (IgG - chronic in SLE an dCLL or with drugs) – Cold agglutinin (IgM - acute in CLL, Mycoplasma pneumonia, Mono) – Coombs positive

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28
Q

Coombs tests

A

Direct is checking for antibody on RBC – indirect is checking for antibody in patient’s serum

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29
Q

Microangiopathic anemia

A

RBCs damaged when passing through obstructed vessel – DIC, TTP/HUS, SLE, malignant hypertension — schistocytes

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30
Q

Macroangiopathic anemia

A

Prosthetic heart valves and aortic stenosis – schistocytes

31
Q

Corticosteroid cell effects

A

Increase neutrophils, decrease lymphocytes and eosinophils

32
Q

Acute intermittent porphyria

A

Deficient porphobilinogen deaminase – accumulation of porphobilinogen, d-ALA – 5Ps (Painful abdomen, Port wine colored urine, Polyneuropathy, Psychological issues, Precipitated by drugs (cyp450 inducers, alcohol) – Tx: Glucose and heme

33
Q

Porphyria cutanea tarda

A

Uroporphyrinogen decarboxylase deficiency – accumulate uroporphyrin (tea colored urine) – blistering cutaneous photosensitivity, red teeth, super hairy

34
Q

Heme pathway diseases

A

GAP UP (substrates - Glycine, Aminolevulinic acid, Porphobilinogen, Uroporphyrinogen, Protoporphyrin) = Some Doctors Prescribe Unlimited Ferrochelatase (enzymes - Synthase, Dehydratase, Porphobilinogen deaminase, Uroporphyrinogen decarboxyalse, Ferrochetalase) = SLAP Lead (diseases - Sideroblastic anemia, Lead poisoning, Acute intermittent porphyria, Porphyria cutanea tarda, Lead poisoning)

35
Q

Iron poisoning

A

Cell death due to peroxidation of membrane lipids – Tx: chelation (deferoxamine, deferasirox)

36
Q

Hemophilias

A

Increased PTT, normal PT – A (VIII - XR), B (IX - XR), C (XI - AR) — macrohemorrhage, hemarthroses, easy bruising, bleeding after trauma — Tx: desmopressin (releases factor 8 and vWF from endothelium) plus missing factor concentrate

37
Q

Vitamin K deficiency

A

Increased PT and PTT but normal bleedign time — decreased factors 2, 7, 9, 10, protein C/S

38
Q

Platelet disorders

A

Increased bleeding time and microhemorrhage (mucosal bleeding, epistaxis, petechia)

39
Q

Bernard Soulier syndrome

A

Increased BT, large platelets – decrased GpIb (defect in platelet to vWF adhesion)

40
Q

Glanzmann thrombasthenia

A

Increased BT – decreased GpIIb/IIIa (defect in platelet to platelet aggregation) – agglutination with ristocetin cofactor assay

41
Q

Immune thrombocytopenia

A

Decreased platelet count, increased BT – anti-GpIIb/IIIa antibodies (viral illness) – increased megakaryocytes – Tx: steroids, IV Immunoglobulin

42
Q

Thrombotic thrombocytopenic purpura

A

Decreased platelet count, increased BT – deficient ADAMST13 (decreased degradation of vWF multimers) – increased large vWF multimers and increased platelet aggregation/thrombosis – schistocytes, increased LDH — neurologic and renal symptoms, fever, thrombocytopenia, microangiopathic hemolytic anemia – Tx: plasmapheresis, steorids

43
Q

von Willebrand disease

A

Increased BT and PTT – lifelong history of mucosal bleeding – autosomal dominant – Ristocetin cofactor assay (decreased agglutination) – Tx: desmopressin

44
Q

DIC

A

Decreased platelet count, increased BT/PT/PTT, increased D-dimers (fibrin split products), schistocytes, decreased fibrinogen, decreased factors V and VIII — deficiency in clotting factors — STOP Making New Thrombi (Sepsis, Trauma, Ob complections, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion, and snake bite)

45
Q

Antithrombin deficiency

A

Diminishes increase in PTT following heparin

46
Q

Factor V leiden

A

Resistant to degradation by activated protein C – DVT, pregnancy loss, cerebral vein thrombosis

47
Q

Protein C or S deficiency

A

Increased risk of thrombotic skin necrosis with hemorrhage following warfarin administration – Protein C Cancels Coagulation

48
Q

Prothrombin gene mutation

A

Increased plasma levels or prothrombin and venous clots

49
Q

Packed RBCs

A

Increase 1Hb/unit – acute blood loss, severe anemia

50
Q

Platelets

A

Stop significant bleeding

51
Q

Fresh frozen plasma

A

Immediate warfarin reversal, cirrhosis, DIC

52
Q

Cryoprecipitate

A

Fibrinogen, factors 8, 13, vWF, fibronectin — coagulation deficiencies involving fibrinogen and factor 8

53
Q

Leukemoid reaction

A

Increased LAP – HUGE WBC count, left shift, lots of neutrophils

54
Q

Hodgkin lymphoma

A

Single group of nodes, contiguous spread, young adults and > 55, associated with EBV – B signs (fever, night sweats, weight loss) — Reed Sternberg cells (CD15/30 positive - owl eyes) – nodular sclerosing form is most common

55
Q

Burkitt lymphoma

A

B cells – young people – t(8;14) c-myc and heavy chain Ig — starry sky (macrophages) – associated with EBV – jaw lesion in Africa, pelvic/adomen in US

56
Q

Diffuse large B cell lymphoma

A

Older adults — MC type in adults – large lymph node or extranodal mass, Waldeyer ring, very agressive

57
Q

Follicular lymphoma

A

MC indolent form, can progress to DLBCL — t(14;18) - BCL-2 (inhibits apoptosis) and Ig heavy chain – painless, waxing and waning lymphadenopathy — Tx: Rituximab

58
Q

Mantle cell lymphoma

A

B cells - Older males – t(11;14) - cyclin D1 (G1 -> S) and heavy chain Ig — CD5+

59
Q

Adult T cell lymphoma

A

Caused by HTLV – cutaneous lesions, lytic bone lesions, hypercalcemia

60
Q

Mycosis fungoides/Sezary syndrome

A

Skin patches/plaques (cutaneous T cell lymphoma), atypical CD4+ T cells — Sezary syndrome when it gets into circulation (T cell leukemia)

61
Q

Multiple myeloma

A

Plasma cell cancer that makes a lot of IgG or IgA — people > 40-50 — increased infections, punched out lytic bone lesions, M spike, Ig light chains in urine (Bence Jones protein), Rouleaux formation — CRAB (hyperCalcemia, Renal involvement, Anemia, Back pain/Bone lytic lesions)

62
Q

Monoclonal gammopathy of undetermined significant

A

Monoclonal expansion of plasma cells, asymptomatic, no CRAB findings

63
Q

Waldenstrom macroglobulinemia

A

M spike = IgM –> hyperviscosity (blurred vision and Raynauds)

64
Q

Acute lymphoblastic leukemia

A

KIDS – T cell can present as mediastinal mass (SVC like) — DOWN SYNDROME! — INCREASED LYMPHOBLASTS — TdT+, CD 10+, Responds well to methotrexate – t(12;21) has best prognosis

65
Q

Small lymphocytic lymphoma/chronic lymphoblastic leukemia

A

Adults > 60 (most common) – CD20+, CD5+ – SMUDGE CELLS – Tx: Prednisone, Rituximab

66
Q

Hairy cell leukemia

A

Adults, hair like projections on cells — dry tap on aspiration – TRAP POSITIVE (tartrate resistant acid phosphatase) - old men with pancytopenia and splenomegaly — Tx: Cladribine, pentostatin

67
Q

Acute myelogenous leukemia

A

Age 15-59 — AUER RODS!!!!!!! – Myeloperoxidase +, LOTS of circulating myeloblasts — t (15;17) - M3 AML subtpye (Tx: ATRA causes differentiation of myelobalsts, DIC complication of treatment) — Tx: Cytarabine

68
Q

Chronic myelogenous leukemia

A

Age 30-59 — PHILADELPHIA CHROMOSOME t(9;22) BCR-ABL – increased neutrophils, metamyeloblasts, BASOPHILIA – very low LAP – Tx: Imatinib

69
Q

Langerhans cell histiocytes

A

Lytic bone lesions and skin rash in child or recurrent otitis media with mass — S-100 stain and Cd1a – Birbeck granules (tennis rackets)

70
Q

Chronic myeloproliferative disorders

A

JAK2 (cytoplasmic tyrosine kinase) mutation — activates JAK-STAT pathway

71
Q

Polycythemia vera

A

Increased hematocrit (increased RBCs/WBCs/platelets) – decreased EPO due to negative feedback – Intense itching after hot shower – erythromelalgia (burning pain with red/blue color) due to blood clots in vessels of extremities — Tx: phlebotomy/hydroxyurea

72
Q

Essential thrombocytosis

A

Overproduction of abnormal platelets – enlarged megakaryocytes

73
Q

Myelofibrosis

A

Increased fibrosis in bone marrow – TEARDROP RBCs – dry tap – massive splenomegaly — Tx: Ruxolitinib

74
Q

Polycythemias

A

Relative (decreased plasma volume d/t deyhdration/burns) — appropriate absolute (increasd RBC mass, decreased O2 saturation, increased EPO - lung/heart disease) — inappropriate absolute (increased RBC mass, increased EPO - kidney/liver cancer due to ectopic EPO) — vera (increased plasma volume, huge increased RBC mass, decreased EPO)

Heme-Onc (3 decks)

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