Heme:Onc Path Flashcards
1
Q
- Symptoms: Leukocoria, increased risk of osteosarcoma. Lab Values: *13q RB tumor supressor gene deletion. Pseudorosettes. * Pathophysiology: *Autosomal dominant. RB regulates G1 to S transition.
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Retinoblastoma
2
Q
- Symptoms: Abdominal mass; myelopathy (spinal cord compression), treatment resistant diarrhea (VIP secretoin), Horner’s syndrome, opsoclonus myoclonus syndrome, ataxia hypertension. Lab Values: 1p N-myc oncogene amplification. HVA (a breakdown product of dopamine) in urine. Pseudorosettes. Bombesin positive. Pathophysiology: The most common tumor of the adrenal medulla in children. Can occur anywhere along the sympathetic chain. Treatment: *Cyclophosphamide
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Neuroblastoma
3
Q
- Symptoms: Granulomatous vasculitis with eosinophilia. Most often presents with ASTHMA, sinusitis, skin lesions, and peripheral neuropathy (e.g., wrist/foot drop, extreme pain); can also involve heart, GI, and kidneys.* Lab Values: 17p p53 tumor supressor gene deletion. Pathophysiology: *Autosomal dominant
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Li-Fraumeni syndrome
4
Q
- Symptoms: Breast and ovarian cancer Lab Values: 17q/13q tumor supressor gene deletion. Pathophysiology: *Autosomal dominant
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BRCA1/2
5
Q
- Symptoms: Colon becomes covered with adenomatous polyps after puberty. Progresses to colon cancer unless resected. Associated with osteomas. Lab Values: 5q APC tumor supressor deletion. Pathophysiology: Autosomal dominant Treatment: *
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Familial adenomatous polyposis
6
Q
- Symptoms: Female with menstural bleeding, uterine leiomyoma (fibroids). Male with GI bleed. Dysphagia, koilonychia (spoon fingernails). Lab Values: *Microcytic, hypochromatic. Decreased serum iron, decreased ferritin, increased TIBC.
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Iron deficiency anemia
7
Q
- Symptoms: Asians or Africans. 1-2 deletions asymptomatic. 3 deletions results in HbH disease. 4 deletions results in hydrops fetalis. Lab Values: Microcytic, hypochromatic. Target cells. Pathophysiology: *HbH = 4 beta chains; Hb Barts = 4 gamma chains
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Alpha-thalassemia
8
Q
- Symptoms: Mediterraneans. Heterozygote asyptomatic. Homozygote has severe anemia. Bone marrow expansion leads to Chipmunk facies (skeleton deformities). Lab Values: Microcytic, hypochromatic. “Crew cut” appearance of skull on X-ray. Increased HbA2 and HbF. Target cells. Pathophysiology: Point mutations in splicing sites and promoter sequences. Repeat transfusions leads to secondary hemochromatosis. Treatment: *Transfusions
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Beta-thalassemia
9
Q
- Symptoms: Microcytic anemia, GI and kidney disease. Mental deterioration in children and memory loss & demyelination in adults. Lab Values: Microcytic, hypochromatic. Basophilic stippling. Increased protoporphyrins. Pathophysiology: *Inhibits ferrochelatase and ALA dehydratase leading to decreased heme synthesis.
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Lead poisoning
10
Q
- Symptoms: Skin paleness, fatigue, dizziness and hepatosplenomegaly. Heart disease, liver damage and kidney failure can result from iron buildup. Lab Values: Microcytic, hypochromatic. Increased serum iron, normal TIBC, increased ferritin. Ringed sideroblasts (iron laden mitochondria). Pathophysiology: X-linked defect in delta-aminolevulinic acid synthase or due to lead/alcohol. Treatment: *Pyridoxine (B6) therapy
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Sideroblastic anemia
11
Q
- Symptoms: Megaloblastic anemia, glossitis. Lab Values: *Macrocytic, hyperchromatic. Hypersegmented neutrophils, increased homocysteine, normal methylmalonic acid.
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Vitamin B9 (Folate) deficiency
12
Q
- Symptoms: Megaloblastic anemia, glossitis, subacute combined degeneration. Lab Values: Macrocytic, hyperchromatic. Hypersegmented neutrophils, increased homocysteine, increased methylmalonic acid. Pathophysiology: *Caused by Diphyllobothrium latum, Crohn’s disease, pernicious anemia, gastrectomy, malnutrition.
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Vitamin B12 deficiency
13
Q
- Symptoms: ** Lab Values: *Macrocytic, hyperchromatic. * Pathophysiology: *Caused by liver disease, alcoholism, reticulocytosis, metabolic disorder (orotic aciduria), 5-FU, AZT, hydroxyurea
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Nonmegaloblastic macrocytic anemia
14
Q
- Symptoms: ** Lab Values: Normocytic, normochromatic. Decreased iron, decreased TIBC, increased ferritin (acute phase protein!) Pathophysiology: *Chronic inflammation elevates hepcidin leading to decreased iron release from macrophages.
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Anemia of chronic disease
15
Q
- Symptoms: No hepatosplenomegaly. Pancytopenia characterized by severe anemia, neutropenia, and thrombocytopenia. Lab Values: Normal cell morphology, but hypocellular bone marrow with fatty infiltration. Pathophysiology: *Caused by radiation, drugs (chloramphenicol, benzene, alkylating agents), viruses (parvovirus B19, EBV, HIV), Fanconi’s anemia, acute hepatitis.
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Aplastic anemia
16
Q
- Symptoms: Small round RBCs with no central pallor. Splenomegaly, aplastic crisis with B19. Lab Values: Normocytic, normochromatic. Increased MCHC, increased RDW. Positive osmotic fragility test. Pathophysiology: Defect in RBC membrane skeleton proteins (ankyrin, band 3.1, spectrin). Increased Howell-Jolly bodies after splenectomy. Treatment: *Splenectomy
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Hereditary spherocytosis
17
Q
- Symptoms: Hemolytic anemia following oxidant stress (sulfas, infections, fava beans). Lab Values: Normocytic, normochromatic. Heinz bodies and bite cells. Pathophysiology: *X-linked recessive. Decreased gluthione synthesis.
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G6PD deficiency
18
Q
- Symptoms: Hemolytic anemia in a newborn. Lab Values: Normocytic, normochromatic. Rigid RBCs due to decreased ATP. Pathophysiology: Autosomal recessive. Treatment: *
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Pyruvate kinase deficiency
19
Q
- Symptoms: Newborns asymptomatic (due to HbF). Salmonella osteomyelitis, autosplenectomy, aplastic crisis (B19), avascular necrosis, resistance to malaria. Non-healing ulcers. Lab Values: Normocytic, normochromatic. “Crew cut” skull on x-ray due to bone marrow expansion. Decreased serum haptoglobin. Decreased ESR. Systolic ejection murmur at right 2nd intercostal space. Pathophysiology: Autosomal recessive. Misense mutation (substitution of normal glutamic acid with valine). Treatment: *Hydroxyurea
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Sickle cell anemia
20
Q
- Symptoms: Mild sickle cell anemia when combined with HbS (HbS/C) Lab Values: *Normocytic, normochromatic.
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HbC defect
21
Q
- Symptoms: Intravascular hemolysis (blood in morning void), hepatic vein thrombosis, abdominal pain. Lab Values: Ham’s (sucrose) test. Increased urine hemosiderin. Pathophysiology: *Complement-mediated hemolysis due to impaired synthesis of GPI anchor/decay-accelerating factor (CD55/CD59)
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Paroxysmal nocturnal hemoglobinuria
22
Q
- Symptoms: Widespread activation of clotting leads to a deficiency in clotting factors, which creates a bleeding state. Lab Values: Schistocytes (helmet cells), increased LDH. Decreased PC and fibrinogen. Increased BT, PT, PTT, TT, d-dimers. Pathophysiology: Caused by sepsis, trauma, obstetric complications (placenta is rich in tissue factor), acute pancreatitis, malignancy, transfusion. Treatment: *Dialysis.
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Diffuse intravascular clotting (DIC)
23
Q
- Symptoms: Microthrombosis, miroangiopathic hemolytic anemia, neurologic symptoms, renal symptoms, thrombocytopenia, fever. Lab Values: Decreased PC, increased BT. Normal PT, PTT, and TT. Pathophysiology: *Deficiency of ADAMTS 13 (vWF metalloprotease).
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Thrombotic thromocytopenic purpura (TTP)
24
Q
- Symptoms: Ingestion of raw meat followed by bloody diarrhea. Renal failure, microangiopathic hemolytic anemia, microthrombosis, less pronounced neurological symptoms. Lab Values: Schistocytes (helmet cells), increased LDH. Decreased PC, increases BT. Normal PT, PTT, and TT. Pathophysiology: *O157:H7 (enterohemorrhagic) E. coli.
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Hemolytic uremic syndrome (HUS)
25
Q
- Symptoms: Epistaxis, petechia, purpura. Lab Values: Decreased PC, increased BT. Normal PT, PTT, and TT. Pathophysiology: *Defect in Gp1b
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Bernard-Soulier disease
26
Q
- Symptoms: Epistaxis, petechia, purpura. Lab Values: Normal PC, increased BT. Normal PT, PTT, and TT. Pathophysiology: Defect in GpIIb/IIIa Treatment: *
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Glanzmann’s thrombasthenia
27
Q
- Symptoms: Epistaxis, petechia, purpura following an acute viral infection. Lab Values: Megakaryocytosis. Deceased PC, increased BT. Normal PT, PTT, and TT. Pathophysiology: *Autoimmune; anti-GpIIb/IIIa antibodies leading to platelet destruction.
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Idiopathic thrombocytopenic purpura (ITP)
28
Q
- Symptoms: Hemarthrosis, bleeding on circumcision, epistaxis, bleeding on cutting teeth. Lab Values: Normal PC. Increased PTT. Normal BT, PT, TT. Pathophysiology: *Deficiency of Factor VIII. X-linked recessive.
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Hemophilia A
29
Q
- Symptoms: Hemarthrosis, bleeding on circumcision, epistaxis, bleeding on cutting teeth. Lab Values: Normal PC. Increased PTT. Normal BT, PT, TT. Pathophysiology: *Deficiency of Factor IX. X-linked recessive.
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Hemophilia B
30
Q
- Symptoms: Neonate with hemorrhage, warfarin use, or following broad-spectrum antibiotics in adult. Lab Values: Decreased factors II, VII, IX, X, protein C, and protein S. Increased PT, PTT. Normal BT Pathophysiology: *Neonates have sterile intestines and are unable to synthesize vitamin K.
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Vitamin K deficiency
31
Q
- Symptoms: Mild. Epistaxis, bleeding following dental procedures. Most common bleeding disorder. Lab Values: Increased PTT (due to decreased factor VIII), increased BT. Normal PT, TT, PC. Failure to aggregate with ristocetin. Pathophysiology: Autosomal dominant. Treatment: *Desmopressin (DDAVP).
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von Willebrand’s disease
32
Q
- Symptoms: Kidney failure Lab Values: *Increased BT. Normal PT, PTT, TT, PC.
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Uremia
33
Q
- Symptoms: Hypercoagulability leading to DVTs and pulmonary embolism. Lab Values: Low PTT that is not corrected with addition of Protein C Pathophysiology: *Mutated factor V is resistant to degradation by protein C.
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Factor V Leiden
34
Q
- Symptoms: Hypercoagulability leading to DVTs and pulmonary embolism. Lab Values: ** Pathophysiology: *Gene mutation in prothrombin 20210A, leading to increased gene expression of prothrombin.
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Prothrombin gene mutation
35
Q
- Symptoms: Hypercoagulability leading to DVTs and pulmonary embolism. Lab Values: Reduced increase in PTT after administration of heparin. Pathophysiology: ** Treatment: *
A
Antithrombin III deficiency
36
Q
- Symptoms: Hypercoagulability leading to DVTs and pulmonary embolism. Hemorrhagic skin necrosis following administration of warfarin. Lab Values: ** Pathophysiology: *Decreased inactivation of Va and VIIIa.
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Protein C or S deficiency
37
Q
- Symptoms: Neonatal hemolysis due to Rh or other blood group incompatiblity; mother’s antibodies attack fetal RBCs. Lab Values: *Combs positive
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Erythroblastosis fetalis
38
Q
- Symptoms: Autoimmune hemolytic anemia Lab Values: Combs positive Pathophysiology: *IgG antibodies. Due to SLE, CLL, or methyldopa
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Warm aggultinins
39
Q
- Symptoms: Autoimmune hemolytic anemia Lab Values: Combs positive Pathophysiology: *IgM antibodies. Due to Mycoplasma pneumoniae, infectious mononucleosis (CMV, EBV).
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Cold agglutinins
40
Q
- Symptoms: Thromobocytopenia, hypercoagulability following heparin administration. Lab Values: ** Pathophysiology: *Autoimmune disease. Antibodies to platelet factor IV-heparin complex.
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Heparin induced thrombocytopenia
41
Q
- Symptoms: Excellent prognosis. Localized, contiguous lymph node group. Fever, night sweats, weight loss. Lab Values: *Collagen banding with lacunar cells. CD15+, CD30+.
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Nodular sclerosis Hodgkin’s lymphoma
42
Q
- Symptoms: Intermediate prognosis. Localized, contiguous lymph node group. Fever, night sweats, weight loss. Lab Values: *Reed-Sternberg cells (CD15+, CD30+). EBV infection.
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Mixed cellularity Hodgkin’s lymphoma
43
Q
- Symptoms: Excellent prognosis. Localized, contiguous lymph node group. Fever, night sweats, weight loss. Lab Values: *Reed-Sternberg cells (CD15-, CD30-, CD45+)
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Lymphocyte predominant Hodgkin’s lymphoma
44
Q
- Symptoms: Poor prognosis. Localized, contiguous lymph node group. Fever, night sweats, weight loss. Lab Values: *Reed-Sternberg cells (CD15+, CD30+). EBV infection.
A
Lymphocyte depleted Hodgkin’s lymphoma
45
Q
- Symptoms: Young child with mandibular or abdomen mass. Lab Values: *t(8:14), CD10+, C19+, CD20+. Starry sky appearance. EBV association. C-myc and heavy-chain Ig
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Burkitt’s lymphoma
46
Q
- Symptoms: Older adults. Most common adult NHL. Lab Values: *Large anaplastic B-cells. CD10+, C19+, CD20+, bcl-6.
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Diffuse large B-cell lymphoma
47
Q
- Symptoms: Older males. Lab Values: *t(11:14), CD5+, CD23-.
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Mantle cell lymphoma
48
Q
- Symptoms: Adults. Lab Values: *t(14:18), bcl-2. CD10+, C19+, CD20+.
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Follicular lymphoma
49
Q
- Symptoms: Associated with chronic inflammatory disease, peptic ulcer disease (H. pylori). Lab Values: CD10+, C19+, CD20+. Pathophysiology: ** Treatment: *Bismuth, metronidazole, ampicillin.
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Marginal zone lymphoma
50
Q
- Symptoms: Adults in Japan and Carribean. Cutaneous lesions/rash, lymphadenopathy, and lytic bone lesions with hypercalcemia. Aggressive. Lab Values: HTLV-1 association. CD3+,CD5+,CD4+. Pathophysiology: *Malignancy of helper T-cells.
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Adult T-cell lymphoma
51
Q
- Symptoms: Adults. Cutaneous patches and nodules. Indolent. Lab Values: CD3+,CD4+,CD5+ Pathophysiology: *Malignancy of helper T-cells.
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Mycosis fungoides/Sezary syndrome
52
Q
- Symptoms: Back pain, lytic bone lesions, primary amyloidosis, anemia, increased infection susceptibility, renal failure. Lab Values: Hypercalcemia, monoclonal IgG spike (kappa light chain), rouleaux formations. “Punched-out” appearance of skull. Bence-Jones protein. Pathophysiology: *Plasma cell neoplasm.
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Multiple myeloma
53
Q
- Symptoms: Hyperviscosity syndrome, no lytic bone lesions. Lab Values: Monoclonal IgM spike.
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Waldenström’s macroglobulinemia (lymphoplasmacytic lymphoma)
54
Q
- Symptoms: Asymptomatic. Lab Values: *Monoclonal IgG spike.
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Monoclonal gammopathy of undetermined significance
55
Q
- Symptoms: *
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Acute lymphoblastic leukemia
56
Q
- Symptoms: >60. #1 cause of non-tender lymphadenopathy. Warm antibody autoimmune hemolytic anemia. More peripheral lymphocytosis than SLL. Increased risk of infections. Lab Values: *Smudge cells. CD5+, CD23+, CD10+, CD19+, CD20+.
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Chronic lymphocytic leukemia
57
Q
- Symptoms: >60. Warm antibody autoimmune hemolytic anemia. Increased risk of infections. Lab Values: *Smudge cells. CD5+, CD23+, CD10+, CD19+, CD20+
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Small lymphocytic lymphoma
58
Q
- Symptoms: Elderly. Increased risk of infections. Lab Values: *Hair like extensions. TRAP positive (tartrate-resistant acid phosphatase). CD19+, CD20+.
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Hairy cell leukemia
59
Q
- Symptoms: 60. Increased DIC risk. Increased risk of infections. Lab Values: Auer rods. M1 t(9:22), M2 t(8:21), M3 t(15:17), M4 inv(16). CD13+, CD33+, MPO+. 20%+ blasts on peripheral smear. Pathophysiology: ** Treatment: *All-trans retinoic acid for M3 variant.
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Acute myelogenous leukemia
60
Q
- Symptoms: 30-60. Increased risk of infections. Splenomegaly. Lab Values: t(9:22), CD13+, CD33+, MPO+, Philadelphia chromosome; ABL-BCR tyrosine kinase. Low leukocyte alkaline phosphatase. Pathophysiology: May progress into a blast crisis (AML). Treatment: *Imatinib
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Chronic myelogenous leukemia
61
Q
- Symptoms: Seborrhea, eosinophilia, lytic skull lesions, diabetes insipidus. Lab Values: Birbeck granules (tennis racket), CD-1a, S-100 Pathophysiology: Monocyte (dendritic cell) neoplasm. Treatment: *
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Langerhans cell histiocytosis
62
Q
- Symptoms: ** Lab Values: *Decrease plasma volume, normal RBC mass and O2 saturation.
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Relative polycythemia
63
Q
- Symptoms: ** Lab Values: Normal plasma volume, increased RBC mass, decreased O2 saturation. Pathophysiology: *Associated with lung disease, congenital heart disease, high altitude.
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Appropriate absolute polycythemia
64
Q
- Symptoms: ** Lab Values: Normal plasma volume, increased RBC mass, normal O2 saturation. Pathophysiology: *Renal cell carcinoma, pheochromocytoma, hemangioblastoma, Wilm’s tumor, cyst, hepatocellular carcinoma, hydronephrosis. Due to ectopic erythropoietin.
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Inappropriate absolute polycythemia
65
Q
- Symptoms: Hypertension, headache, cyanosis, thrombosis. Pruritus following a hot shower because of histamine release from basophils. Lab Values: *JAK2 mutation. Low erythropoietin. Elevated hematocrit (~60%), elevated hemoglobin (~20g/dL). Decreased ESR. Increased plasma volume, increased RBC mass, normal O2 saturation.
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Polycythemia vera
66
Q
- Symptoms: Asymptomatic. Lab Values: JAK2 mutation. 800,000-1,000,000+ PC. Megakaryocytosis. Pathophysiology: ** Treatment: *
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Essential thrombocytosis
67
Q
- Symptoms: Increased infection risk. Caused by either bone marrow burn out or metastatic carcinoma. Lab Values: Teardrop cells. JAK2 mutation. Leukoerythroblastosis (nucleated RBCs). Fibrotic obliteration of bone marrow. Pathophysiology: *May progress into a blast crisis (AML).
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Myelofibrosis, myelophthisic anemia
68
Q
- Symptoms: ** Lab Values: *Sideroblasts, hyposegemented neutrophils. Pancytopenia, atypical leukocytes.
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Myelodysplastic syndrome
69
Q
- Symptoms: *Dysphagia (esophageal webs), glossitis, iron deficiency anemia. Leads to esophageal squamous cell carcinoma.
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Plummer-Vinson syndrome
70
Q
- Symptoms: Infection Lab Values: *Looks like CML except that it has a HIGH leukocyte alkaline phosphatase level
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Leukemoid reaction
71
Q
- Symptoms: *Thymoma, leukemias
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Pure red cell aplasia (erythrocytopenia)
72
Q
- Symptoms: Associated with EBV infection
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Nasopharyngeal carcinoma
73
Q
- Symptoms: Hypercoagulation, miscarriages. Associated with SLE. Lab Values: *Elevated PTT despite the addition of clotting factors. False positive VLDR.
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Antiphospholipid syndrome
74
Q
- Symptoms: *Cutaneous necrosis associated with Pseudomonas speticemia. Usually a neutropenic or burn patient.
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Ecthyma gangrenosum
75
Q
- Symptoms: *Hepatocellular carcinoma
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Aflatoxins (Aspergillus)
76
Q
- Symptoms: *Centrilobular necrosis of liver, fatty change.
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CCL4
77
Q
- Symptoms: *Gastric cancer; seen in smoked foods.
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Nitrosamines
78
Q
- Symptoms: Bronchogenic carcinoma. Requires CYP-450 activation in liver. Lab Values: ** Pathophysiology: ** Treatment: *
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Benzopyrene
79
Q
- Symptoms: Bronchogenic carcinoma»_space;> malignant mesothelioma Lab Values: ** Pathophysiology: ** Treatment: *
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Absestos
80
Q
- Symptoms: *Bronchogenic carcinoma
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Chromium and nickle
81
Q
- Symptoms: Squamous cell carcinoma of the skin and lung, angiosarcoma of the liver
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Arsenic
82
Q
- Symptoms: *Angiosarcoma of liver
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Vinyl chloride
83
Q
- Symptoms: *Hepatocellular carcinoma
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Aromatic amines and azo dyes
84
Q
- Symptoms: *Leukemia, lymphoma
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Alkylating agents
85
Q
- Symptoms: *Leukemia
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Benzene
86
Q
- Symptoms: Transitional cell carcionma
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Napthylamine
87
Q
- Symptoms: *Superficial vascular proliferation due to Bartonella henselae infection in HIV patients.
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Bacillary angiomatosis
88
Q
- Symptoms: Micronodular cirrhosis, diabetes mellitus, and skin pigmentation; “bronze diabetes.” Results in congestive heart failure and increased risk of hepatocellular carcinoma. Lab Values: Increased serum iron, decreased transferrin/TIBC, increased ferritin, greatly increaseed % transferrin saturation. HLA-A3. Pathophysiology: Autosomal recessive (primary) or secondary to chronic transfusion therapy. Deposition of hemosiderin in the periphery. Treatment: *Deferoxamine, phelbotomy.
A
Hemochromatosis
89
Q
- Symptoms: ** Lab Values: Prolonged PT and PTT. Normal BT and PC. Pathophysiology: Factor X deficiency. Treatment: *
A
Amyloid purpura
90
Q
- Symptoms: Squamous cell carcinoma (larynx/lung), small cell carcinoma (lung), renal cell carcinoma, transitional cell carcinoma (bladder). Lab Values: ** Pathophysiology: ** Treatment: *
A
Cigarette smoke
91
Q
- Symptoms: Acute onset left-sided abdominal pain, pallor, hypotension, tachycardia, shortness of breath. Lab Values: Anemic, low PC, low hematocrit. Pathophysiology: ** Treatment: *
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Splenic sequestration
92
Q
- Symptoms: ** Lab Values: Low C4 and C3. Pathophysiology: Deficiency in C1 esterase inhibitor. Treatment: *Danazol.
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Hereditary angioedema
93
Q
- Symptoms: Fibrin deposits along mitral valve. Lab Values: ** Pathophysiology: Associated with patients who have cancer; cachexia, wasting diseases. Treatment: *
A
Nonbacterial thrombotic endocarditis (marantic)
94
Q
- Symptoms: Unilateral limb swelling, warmth, erythema; tends to be leg (iliac, femoral, popliteal). Lab Values: Homan sign (increased resistance to passive dorsiflexion of the ankle). Detected by doppler “duplex” ultrasound. Pathophysiology: May become a pulmonary embolus or can go to the brain if an ASD/VSD is present. Treatment: *
A
Deep venous thrombosis
95
Q
- Symptoms: Hypercoagluable state. Acrocyanosis, retinal hemorrhage, Raynaud’s phenomenon with digital ulceration, livedo reticularis, purpura, arthralgia (PIP, MCP). Lab Values: ** Pathophysiology: Associated with Hepatitis C or chronic inflammatory states (SLE, RA). Can cause RPGN and MPGN. Treatment: *
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Cryoglobulinemia
96
Q
- Symptoms: Thrombocytopenia (or low hematocrit) due to overactive spleen in an alcoholic. Lab Values: Low PC, high BT. Pathophysiology: Portal hypetension due to hepatic cirrhosis leads to congestion in the spleen. This results in overactivity and sequestration of platelets and RBCs. Treatment: *
A
Hypersplenism
97
Q
- Symptoms: Multiple aphthous ulcers, genital ulcers, and uveitis. Lab Values: ** Pathophysiology: Immune complex vasculitis associated with viral infections. Treatment: *
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Behcet’s syndrome
98
Q
- Symptoms: *Infant (
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Letterer-Siwe disease
99
Q
- Symptoms: Pathological bone fracture in an adolescent; no skin involvement. Lab Values: Langerhans cells with eosinophils. Pathophysiology: Benign proliferation of histiocytes. Treatment: *
A
Eosinophilic granuloma
100
Q
- Symptoms: Child (>3 y/o) with diabetes insipidus, exopthalmos, lytic skull defets, scalp rash. Lab Values: ** Pathophysiology: Malignant proliferation of histocytes. Treatment: *
A
Hand-Schuller-Christian disease
101
Q
- Symptoms: Symptoms of DIC without elevated D-dimers Lab Values: Elevated PT/PTT/BT, decreased PC, with NORMAL D-DIMERS**.* Pathophysiology: Caused by cirrhosis (lack of antiplasmin-2alpha) or radical prostatectomy (release of tPA). Treatment: *Epsilon-aminocaproic acid
A
Fibrinolysis defect
