Heme:Onc Path Flashcards by Jacob Townsend

Symptoms: Leukocoria, increased risk of osteosarcoma. Lab Values: *13q RB tumor supressor gene deletion. Pseudorosettes. * Pathophysiology: *Autosomal dominant. RB regulates G1 to S transition.

Retinoblastoma

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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

Neuroblastoma

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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

Li-Fraumeni syndrome

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Symptoms: Breast and ovarian cancer Lab Values: 17q/13q tumor supressor gene deletion. Pathophysiology: *Autosomal dominant

BRCA1/2

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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: *

Familial adenomatous polyposis

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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.

Iron deficiency anemia

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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

Alpha-thalassemia

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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

Beta-thalassemia

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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.

Lead poisoning

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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

Sideroblastic anemia

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Symptoms: Megaloblastic anemia, glossitis. Lab Values: *Macrocytic, hyperchromatic. Hypersegmented neutrophils, increased homocysteine, normal methylmalonic acid.

Vitamin B9 (Folate) deficiency

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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.

Vitamin B12 deficiency

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Symptoms: ** Lab Values: *Macrocytic, hyperchromatic. * Pathophysiology: *Caused by liver disease, alcoholism, reticulocytosis, metabolic disorder (orotic aciduria), 5-FU, AZT, hydroxyurea

Nonmegaloblastic macrocytic anemia

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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.

Anemia of chronic disease

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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.

Aplastic anemia

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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

Hereditary spherocytosis

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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.

G6PD deficiency

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Symptoms: Hemolytic anemia in a newborn. Lab Values: Normocytic, normochromatic. Rigid RBCs due to decreased ATP. Pathophysiology: Autosomal recessive. Treatment: *

Pyruvate kinase deficiency

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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

Sickle cell anemia

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Symptoms: Mild sickle cell anemia when combined with HbS (HbS/C) Lab Values: *Normocytic, normochromatic.

HbC defect

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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)

Paroxysmal nocturnal hemoglobinuria

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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.

Diffuse intravascular clotting (DIC)

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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).

Thrombotic thromocytopenic purpura (TTP)

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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.

Hemolytic uremic syndrome (HUS)

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* Symptoms: *Epistaxis, petechia, purpura.* Lab Values: *Decreased PC, increased BT. Normal PT, PTT, and TT.* Pathophysiology: *Defect in Gp1b

Bernard-Soulier disease

* Symptoms: *Epistaxis, petechia, purpura.* Lab Values: *Normal PC, increased BT. Normal PT, PTT, and TT.* Pathophysiology: *Defect in GpIIb/IIIa* Treatment: *

Glanzmann's thrombasthenia

* 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.

Idiopathic thrombocytopenic purpura (ITP)

* 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.

Hemophilia A

Hemophilia B

* 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.

Vitamin K deficiency

* 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).

von Willebrand's disease

* Symptoms: *Kidney failure* Lab Values: *Increased BT. Normal PT, PTT, TT, PC.

Uremia

* 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.

Factor V Leiden

* Symptoms: *Hypercoagulability leading to DVTs and pulmonary embolism.* Lab Values: ** Pathophysiology: *Gene mutation in prothrombin 20210A, leading to increased gene expression of prothrombin.

Prothrombin gene mutation

* Symptoms: *Hypercoagulability leading to DVTs and pulmonary embolism.* Lab Values: *Reduced increase in PTT after administration of heparin.* Pathophysiology: ** Treatment: *

Antithrombin III deficiency

* Symptoms: *Hypercoagulability leading to DVTs and pulmonary embolism. Hemorrhagic skin necrosis following administration of warfarin.* Lab Values: ** Pathophysiology: *Decreased inactivation of Va and VIIIa.

Protein C or S deficiency

* Symptoms: *Neonatal hemolysis due to Rh or other blood group incompatiblity; mother's antibodies attack fetal RBCs.* Lab Values: *Combs positive

Erythroblastosis fetalis

* Symptoms: *Autoimmune hemolytic anemia* Lab Values: *Combs positive* Pathophysiology: *IgG antibodies. Due to SLE, CLL, or methyldopa

Warm aggultinins

* Symptoms: *Autoimmune hemolytic anemia* Lab Values: *Combs positive* Pathophysiology: *IgM antibodies. Due to Mycoplasma pneumoniae, infectious mononucleosis (CMV, EBV).

Cold agglutinins

* Symptoms: *Thromobocytopenia, hypercoagulability following heparin administration.* Lab Values: ** Pathophysiology: *Autoimmune disease. Antibodies to platelet factor IV-heparin complex.

Heparin induced thrombocytopenia

* Symptoms: *Excellent prognosis. Localized, contiguous lymph node group. Fever, night sweats, weight loss.* Lab Values: *Collagen banding with lacunar cells. CD15+, CD30+.

Nodular sclerosis Hodgkin's lymphoma

* Symptoms: *Intermediate prognosis. Localized, contiguous lymph node group. Fever, night sweats, weight loss.* Lab Values: *Reed-Sternberg cells (CD15+, CD30+). EBV infection.

Mixed cellularity Hodgkin's lymphoma

* Symptoms: *Excellent prognosis. Localized, contiguous lymph node group. Fever, night sweats, weight loss.* Lab Values: *Reed-Sternberg cells (CD15-, CD30-, CD45+)

Lymphocyte predominant Hodgkin's lymphoma

* Symptoms: *Poor prognosis. Localized, contiguous lymph node group. Fever, night sweats, weight loss.* Lab Values: *Reed-Sternberg cells (CD15+, CD30+). EBV infection.

Lymphocyte depleted Hodgkin's lymphoma

* 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

Burkitt's lymphoma

* Symptoms: *Older adults. Most common adult NHL.* Lab Values: *Large anaplastic B-cells. CD10+, C19+, CD20+, bcl-6.

Diffuse large B-cell lymphoma

* Symptoms: *Older males.* Lab Values: *t(11:14), CD5+, CD23-.

Mantle cell lymphoma

* Symptoms: *Adults.* Lab Values: *t(14:18), bcl-2. CD10+, C19+, CD20+.

Follicular lymphoma

* Symptoms: *Associated with chronic inflammatory disease, peptic ulcer disease (H. pylori).* Lab Values: *CD10+, C19+, CD20+.* Pathophysiology: ** Treatment: *Bismuth, metronidazole, ampicillin.

Marginal zone lymphoma

* 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.

Adult T-cell lymphoma

* Symptoms: *Adults. Cutaneous patches and nodules. Indolent.* Lab Values: *CD3+,CD4+,CD5+* Pathophysiology: *Malignancy of helper T-cells.

Mycosis fungoides/Sezary syndrome

* 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.

Multiple myeloma

* Symptoms: *Hyperviscosity syndrome, no lytic bone lesions.* Lab Values: *Monoclonal IgM spike.*

Waldenström's macroglobulinemia (lymphoplasmacytic lymphoma)

* Symptoms: *Asymptomatic.* Lab Values: *Monoclonal IgG spike.

Monoclonal gammopathy of undetermined significance

* Symptoms: *

Acute lymphoblastic leukemia

* 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+.

Chronic lymphocytic leukemia

* Symptoms: *>60. Warm antibody autoimmune hemolytic anemia. Increased risk of infections.* Lab Values: *Smudge cells. CD5+, CD23+, CD10+, CD19+, CD20+

Small lymphocytic lymphoma

* Symptoms: *Elderly. Increased risk of infections.* Lab Values: *Hair like extensions. TRAP positive (tartrate-resistant acid phosphatase). CD19+, CD20+.

Hairy cell leukemia

* 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.

Acute myelogenous leukemia

* 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

Chronic myelogenous leukemia

* Symptoms: *Seborrhea, eosinophilia, lytic skull lesions, diabetes insipidus.* Lab Values: *Birbeck granules (tennis racket), CD-1a, S-100* Pathophysiology: *Monocyte (dendritic cell) neoplasm.* Treatment: *

Langerhans cell histiocytosis

* Symptoms: ** Lab Values: *Decrease plasma volume, normal RBC mass and O2 saturation.

Relative polycythemia

* Symptoms: ** Lab Values: *Normal plasma volume, increased RBC mass, decreased O2 saturation.* Pathophysiology: *Associated with lung disease, congenital heart disease, high altitude.

Appropriate absolute polycythemia

* 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.

Inappropriate absolute polycythemia

* 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.

Polycythemia vera

* Symptoms: *Asymptomatic.* Lab Values: *JAK2 mutation. 800,000-1,000,000+ PC. Megakaryocytosis.* Pathophysiology: ** Treatment: *

Essential thrombocytosis

* 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).

Myelofibrosis, myelophthisic anemia

* Symptoms: ** Lab Values: *Sideroblasts, hyposegemented neutrophils. Pancytopenia, atypical leukocytes.

Myelodysplastic syndrome

* Symptoms: *Dysphagia (esophageal webs), glossitis, iron deficiency anemia. Leads to esophageal squamous cell carcinoma.

Plummer-Vinson syndrome

* Symptoms: *Infection* Lab Values: *Looks like CML except that it has a HIGH leukocyte alkaline phosphatase level

Leukemoid reaction

* Symptoms: *Thymoma, leukemias

Pure red cell aplasia (erythrocytopenia)

* Symptoms: *Associated with EBV infection*

Nasopharyngeal carcinoma

* Symptoms: *Hypercoagulation, miscarriages. Associated with SLE.* Lab Values: *Elevated PTT despite the addition of clotting factors. False positive VLDR.

Antiphospholipid syndrome

* Symptoms: *Cutaneous necrosis associated with Pseudomonas speticemia. Usually a neutropenic or burn patient.

Ecthyma gangrenosum

* Symptoms: *Hepatocellular carcinoma

Aflatoxins (Aspergillus)

* Symptoms: *Centrilobular necrosis of liver, fatty change.

CCL4

* Symptoms: *Gastric cancer; seen in smoked foods.

Nitrosamines

* Symptoms: *Bronchogenic carcinoma. Requires CYP-450 activation in liver.* Lab Values: ** Pathophysiology: ** Treatment: *

Benzopyrene

* Symptoms: *Bronchogenic carcinoma >>> malignant mesothelioma* Lab Values: ** Pathophysiology: ** Treatment: *

Absestos

* Symptoms: *Bronchogenic carcinoma

Chromium and nickle

* Symptoms: *Squamous cell carcinoma of the skin and lung, angiosarcoma of the liver*

Arsenic

* Symptoms: *Angiosarcoma of liver

Vinyl chloride

* Symptoms: *Hepatocellular carcinoma

Aromatic amines and azo dyes

* Symptoms: *Leukemia, lymphoma

Alkylating agents

* Symptoms: *Leukemia

Benzene

* Symptoms: *Transitional cell carcionma*

Napthylamine

* Symptoms: *Superficial vascular proliferation due to Bartonella henselae infection in HIV patients.

Bacillary angiomatosis

* 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.

Hemochromatosis

* Symptoms: ** Lab Values: *Prolonged PT and PTT. Normal BT and PC.* Pathophysiology: *Factor X deficiency.* Treatment: *

Amyloid purpura

* Symptoms: *Squamous cell carcinoma (larynx/lung), small cell carcinoma (lung), renal cell carcinoma, transitional cell carcinoma (bladder).* Lab Values: ** Pathophysiology: ** Treatment: *

Cigarette smoke

* Symptoms: *Acute onset left-sided abdominal pain, pallor, hypotension, tachycardia, shortness of breath.* Lab Values: *Anemic, low PC, low hematocrit.* Pathophysiology: ** Treatment: *

Splenic sequestration

* Symptoms: ** Lab Values: *Low C4 and C3.* Pathophysiology: *Deficiency in C1 esterase inhibitor.* Treatment: *Danazol.

Hereditary angioedema

* Symptoms: *Fibrin deposits along mitral valve.* Lab Values: ** Pathophysiology: *Associated with patients who have cancer; cachexia, wasting diseases.* Treatment: *

Nonbacterial thrombotic endocarditis (marantic)

* 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: *

Deep venous thrombosis

* 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: *

Cryoglobulinemia

* 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: *

Hypersplenism

* Symptoms: *Multiple aphthous ulcers, genital ulcers, and uveitis.* Lab Values: ** Pathophysiology: *Immune complex vasculitis associated with viral infections.* Treatment: *

Behcet's syndrome

* Symptoms: *Infant (

Letterer-Siwe disease

* Symptoms: *Pathological bone fracture in an adolescent; no skin involvement.* Lab Values: *Langerhans cells with eosinophils.* Pathophysiology: *Benign proliferation of histiocytes.* Treatment: *

Eosinophilic granuloma

* Symptoms: *Child (>3 y/o) with diabetes insipidus, exopthalmos, lytic skull defets, scalp rash.* Lab Values: ** Pathophysiology: *Malignant proliferation of histocytes.* Treatment: *

Hand-Schuller-Christian disease

* 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

Fibrinolysis defect