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PANCE
hematology
| Question | Answer |
|---|---|
| Causes of anemias? | increased red cell destruction, decreased red cell production, bleeding; secondary to a systemic disease |
| What are the hypochromic microcytic anemias? | iron deficiency anemia, thalassemia, sickle-cell anemia, long-standing anemia of chronic disease |
| In microcytic anemias with an MCV < 70 fL, the cause is either ______ _________ or __________. | iron deficiency, thalassemia |
| Macrocytic anemias with an MCV > 125 fL almost always are ________, except for those associated with ____________ ________. | megaloblastic, myelodysplastic syndromes |
| What laboratory studies are typically used to evaluate anemias? | CBC (Hgb, Hct, red cell indices, red cell distribution width), peripheral smear, corrected reticulocyte count |
| What is the most common cause of anemia in the world? | iron deficiency anemia (inadequate supply of iron for synthesis of hemoglobin) |
| What is usually the cause of iron deficiency in adults? | bleeding from the GI tract; chronic ASA or NSAID use may contribute to the cause |
| What are other typical causes of iron deficiency anemia? | low dietary intake of Fe (children and pregnant women), menstrual blood loss |
| Clinical features of iron deficiency anemia? | general complaints (Hct < 25%) include pallor, easy fatigability, irritability, anorexia, tachycardia, DOE, poor weight gain in infants |
| What is a hallmark of iron deficiency? | pica |
| What are clinical features of severe iron deficiency anemia? | brittle nails, cheliosis, smooth tongue, formation of esophageal webs (Plummer-Vinson syndrome) |
| Laboratory findings in iron deficiency anemia? | H&H decreased; eventually peripheral smear shows hypochromic microcytic RBC's, anisocytosis, and poikilocytosis; plasma ferritin and serum Fe both decrease to < 30 ug/dL; TIBC ELEVATED; transferrin sats < 15% |
| Tx of iron deficiency anemia? | 1. ID bleeding source 2. ferrous sulfate, 325 mg tid, given in a slow, escalating dose (best absorbed on empty stomach, but frequently given w/ meals to avoid AE's) 3. parenteral Fe appropriate for certain pt's |
| After starting tx of Fe deficiency anemia, how long will it take for Hct to reach normal levels? How long should supplementation continue? | should reach normal levels within 2 months, but supplementation should continue for up to 6 mo or longer to replenish tissue stores |
| Which patients need iron supplementation? | during pregnancy and lactation |
| hereditary anemias in which synthesis of alpha or beta-globin chains is reduced, resulting in defective hemoglobinization of RBC's | thalassemia syndromes |
| caused by gene deletion; most commonly seen in ppl of Southeast Asian or Chinese origin | alpha-thalassemia |
| caused by point mutations; most commonly seen in Mediterranean populations | beta-thalassemia |
| What is the most common feature of the thalassemias? | microcytosis out of proportion to the degree of anemia |
| Who should thalassemia be suspected in? | in a person w/ positive family hx or a personal hx of lifelong microcytic anemia, esp. if unresponsive to iron therapy |
| Clinical features of alpha-thalassemias? (keep in mind that deficits range from silent carriers to profound anemia) | may have mild sx or none (thal trait; carriers). Pt's often dx'ed after a nonresponse to tx for a prescribed Fe deficiency. |
| Patients with only one alpha-globin chain have ___________ _________, which is variably symptomatic. When all 4 chains are deleted, what occurs? | hemoglobin H disease; stillbirth occurs from hydrops fetalis |
| When do problems begin with beta thalassemia major? | problems begin at 4-6 mo's, when the switch from fetal hemoglobin (hemoglobin F) to adult hemoglobin occurs |
| Clinical features of beta thalassemia major? | severe anemia, growth retardation, abnormal faical structure, pathologic fractures, osteopenia, bone deformities, hepatosplenomegaly, jaundice |
| Labs to obtain if thalassemia is suspected? | CBC, retic count, peripheral smear, HEMOGLOBIN ELECTROPHORESIS CONFIRMS DIAGNOSIS |
| What will peripheral smear show with thalassemias? | marked microcytosis for degree of anemia |
| Hct 28-40%, normal Hgb electrophoresis, normal retic count, peripheral smear showing target cells and acanthocytes | alpha-thalassemia minor (trait) |
| Hct 22-32%, increased retic count, peripheral smear shows target cells and poikilocytes, electrophoresis shows hemoglobin H | alpha-thalassemia H |
| Hct 28-40%, normal or increased retic count, peripheral smear shows target cells and basophilic stippling; electrophoresis shows Hemoglobin A2 and hemoglobin F | B-thalassemia minor |
| Hct as low as 10%, increased retic count, peripheral smear shows target cells, poikilocytes, basophilic stippling, nucleated RBC's; electrophoresis shows hgb F and A2 | B-thalassemia major |
| Treatment of alpha-thalassemia (hgb H) disease? | folic acid supplements and AVOID iron supplements and oxidative drugs (i.e. dapsone, quinidine, sulfonamides) --> risk of iron overload |
| Treatment of B-thalassemia major? | transfusions to keep hgb concentrations at least 12 g/dL; may also be tx'ed w/ allogenic bone marrow transplantation and iron chelation or splenectomy |
| Patients w/ b-thalassemia major used to die fm cardiac failure by age 30 (before effective Fe chelation and allogenic stem cell transplants). What does iron overload cause? | hemosiderosis, heart failure, cirrhosis, endocrinopathies (use deferoxamine to avoid or postpone hemosiderosis) |
| Treatment of mild alpha or beta thalassemia? | do not give Fe d/t risks associated with Fe overload |
| acquired d/o's with reduced Hgb synthesis causing iron accumulation, especially in the mitochondria | sideroblastic anemia |
| Causes of sideroblastic anemia? | myelodysplais, chronic alcoholism, lead poisoning |
| Laboratory findings associated with sideroblastic anemia? | peripheral smear shows NL and hypochromic cells; Prussian blue staining of bone marrow cells shows ringed sideroblasts (BONE MARROW EVAL NECESS FOR DX); Hct 20-30% |
| Treatment of sideroblastic anemia? | tx underlying cause, transfusion may be required |
| anemias caused by organ failure or impaired marrow function resulting from systemic disease; MCV 80-100 fL | normochromic normocytic anemias |
| Significant anemia in pt's with chronic disease usually indicates what kind of anemia? | folate or iron deficiency |
| anemia that arises from injury or abnormal expression of the pluripotent hematopoietic stem cell | aplastic anemia |
| Clinical features of aplastic anemia? | weakness, fatigue, vulnerability to infection, pallor, purpura, petechiae |
| Laboratory studies-hallmark of aplastic anemia? | pancytopenia; bone marrow is hypocellular |
| Treatment of aplastic anemia? | mild-supportive tx with transfusion of RBC's and paltelets; severe-bone marrow transplantation or immunosuppression |
| What causes macrocytic anemias (MCV > 100 fL)? | acute hemorrhage, hemolysis, deficiences leading to megaloblastic states |
| What is the most common cause of folic acid deficiency? | poor dietary intake |
| Other causes of folic acid deficiency? | pregnancy, hemolytic anemia, alcohol abuse, consumption of folic acid antagonists |
| Clinical features of folic acid deficiency? | sore tongue (glossitis), vague GI symptoms; no neuro sx (in contrast to vitamin B12 deficiency) |
| Lab findings of folic acid deficiency? | MACRO-OVALOCYTES AND HYPERSEGMENTED PMNS ARE PATHOGNOMONIC; Howell-Jolly bodies are typical; RBC folate < 150 ng/mL is diagnostic; vit. B12 is NL |
| Treatment of folic acid deficiency? | folic acid 1 mg/day=first line tx DISCONTINUE ALCOHOL AND FOLIC ACID ANTAGONISTS |
| Causes of vitamin B12 (cobalamin) deficiency? | pernicious anemia (most common cause) --> leads to atrophic gastritis and increased risk of gastric carcinoma; strict vegan diet, gastric surgery, Crohn's disease |
| Where does absorption and storage of B12 occur? | absorption in terminal ileum and storage in liver |
| What foods supply vitamin B12? | foods of animal origin |
| What is the most concerning effect of uncorrected B12 deficiency? | irresversible neuro damage can be caused by uncorrected deficiency |
| What can mask, but not correct, vitamin B12 deficiency? | folate supplementation |
| Clinical features of vitamin B12? | glossitis and vague GI symptoms; neuro findings include stocking-glove paresthesias, loss of position, fine touch and vibratory sensation, clumsiness, dementia, and ataxia |
| Laboratory studies for diagnosis of vitamin B12? | anemia may be severe, MCV usually markedly elevated; anisocytosis, poikilocytosis, macro-ovalocytosis, hypersegmented neutrophils; retic count reduced; B12 LOW! |
| Treatment of vitamin B12 deficiency? | lifelong supplemental vitamin B12 usually given IM for pernicious anemia; daily oral cobalamin may be used; neuro signs/sx reversible if treated w/in 6 months |
| What causes hemolytic anemias? | characterized by episodic or continuous RBC destruction; based on intrinsic red cell defects or extracellular causes |
| What are intrinsic causes of hemolytic anemia? | hereditary spherocytosis and elliptocytosis, paroxysmal noctural hemoglobinuria, G6PD deficiency, methemoglobinemia, sickle-cell sydnromes |
| What are external causes of hemolytic anemia? | autoimmune and lymphoproliferative diseases, drug toxicity, thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome, DIC, valvular hemolysis, metastatic adenocarcinoma, vasculitides, infxns, hypersplenism, burns |
| Clinical features of hemolytic anemias? | may present with jaundice, gallstones, pallor, and sx related to decreased O2 delivery to tissues; infection w/ parvovirus B19 can lead to a transient aplastic crisis |
| Lab findings in hemolytic anemia? | elevated retic count, peripheral smear may show immature RBC's, nucleated RBC's, or morphologic changes; indirect bili and total bili slightly elevated |
| Treatment of hemolytic anemias? | treat underlying disorder |
| autosomal recessive hemolytic anemia | sickle cell anemia |
| Pathophysiology of sickle cell anemia? | RBC's containing primarily hemoglobin S sickle under deoxygenated conditions |
| Clinical features of sickle cell anemia? | problems beginin infancy when hemoglobin F levels fall; by childhood or adolescence, vascular occlusions produce painful crises, lasting for hrs to days, with organ swelling and dysfunction and infarction |
| What causes sickling to increase? | dehydration, acidosis, hypoxemia |
| What problems are sickle cell patients at increased risk for? | cholelithiasis, splenomegaly, poorly healing ulcers, infection with encapsulated organisms (ie. strep pneumoniae), strokes, priapism, retinopathies, osteomyelitis, avascular necrosis of femoral head; aplastic crises (life-threatening) |
| Diagnosis of sickle cell anemia? | Hgb electrophoresis shows hemoglobin S in RBC's, peripheral smear reveals sickled cells, target cells, nucleated RBC's, Howell-Jolly bodies, elevated retic count |
| Treatment of sickle cell anemia? | 1. Symptomatic tx includes administration of analgesics, fluids, oxygen. 2. Folic acid supplementation. 3. Hydroxyurea for severe cases. 4. Pneumococcal vaccine 5. Vaso-occlusive may require transfusion |
| X-linked recessive d/o commonly seen in American black males and Mediterranean populations | G6PD deficiency |
| Pathophysiology of G6PD deficiency? | characterised by abnormally low levels of glucose-6-phosphate dehydrogenase, a metabolic enzyme involved in the pentose phosphate pathway, especially important in RBC metabolism |
| What may cause episodic hemolysis in patients with G6PD deficiency? | infection and oxidant drugs (i.e. dapsone, quinidine, nitrofurantoin, sulfonamides) may cause episodic hemolysis |
| Clinical features of patients with G6PD deficiency? | female carriers rarely affected; patients w/ episodic hemolysis usually healthy and w/o splenomegaly |
| Lab findings in patient with G6PD deficiency? | G6PD levels low in between hemolytic episodes (if severe, G6PD levels always low); peripheral smear reveals bite cells and Heinz bodies; during hemolytic episodes, retics and indirect bili increase |
| Treatment of G6PD deficiency? | AVOID oxidative drugs; hemolytic episodes are self-limited as RBC's are replaced |
| diseases characterized by unrestrained growth of leukocytes and leukocyte precursors in the tissues | leukemias |
| How are leukemias classified? | lymphoid and myeloid, acute and chronic |
| Risk factors for leukemia? | positive family history, exposures to ionizing radiation, benzene, certain alkylating agents |
| Who does acute lymphocytic leukemia tend to affect most? | children, ages 3-7 (80%) |
| Who does acute myelogenous leukemia tend to affect most? | median onset is 60 years old |
| Clinical features of acute leukemias? | findings related to replacement of normal bone marrow with malignant cells; gingival bleeding, epistaxis, menorrhagia; infxns fm neutropenia commonly caused by gm-neg bacteria or fungi (common presentations-cellulitis, pneumonia, perirectal infxn) |
| How do children and young adults tend to present with acute leukemias? | fatigue, abrupt onset of fever, lethargy, H/A bone or joint pain |
| How do older adults tend to present with acute leukemias? | slow, progressive onset with lethargy, anorexia, dyspnea |
| Lymphadenopathy and splenomegaly are more common with ____ than ____. | ALL, AML |
| Laboratory findings with acute leukemias? | HALLMARK=pancytopenia w/ circulating blasts (blasts make up at least 20% of nucleated cells in bone marrow); WBC counts usually high, hyperuricemia |
| What confirms the diagnosis of acute leukemias? | bone marrow biopsy |
| What findings are seen specifically with AML? | auer rods (rod-shaped structures in cell cytoplasm) |
| What findings are seen specifically with ALL? | mediastinal mass on CXR, terminal deoxynucleotidyl transferase (present in 95% cases), presence of Philadelphia chromosome unfavorable in ALL |
| Treatment of ALL? | >50% children with ALL can be cured with chemotherapy (induction chemo plus consolidation therapy) |
| Prognosis of ALL related to what factors? | related to age and WBC count at diagnosis |
| Treatment of AML? | greater than 70% of adults younger than 60 years achieve complete remission w/ tx for AML; further chemo leads to cure in 30-40% patients |
| Possible treatments available for AML and ALL? | 1. induction (remission-inducing) chemotherapy targeted toward eradication of most of leukemic cells 2. consolidation therapy destroys remainder of leukemic cells 3. allogeneic bone marrow transplantation used in pt's w/ poor response to tx |
| clonal malignancy of B lymphocytes; most prevalent of all leukemias; 2x common in men as women; median presenting age is 65 y.o. | chronic lymphocytic leukemia (CLL) |
| Normal course of CLL? | usually has indolent course, w/ a median survival time of 6 yrs; often harmless but resistant to cure (a variant, polylymphocytic leukemia, is more aggressive) |
| Clinical manifestations of CLL? | peripheral lymphocytosis and lymphocytic invasion of bone marrow, liver, spleen, and lymph nodes; recurrent infxns, splenomegaly, LA common |
| What syndrome occurs in 5% cases of CLL? | Richter's syndrome --> an isolated node transforms into aggressive, large-cell lymphoma |
| Laboratory findings associated with CLL? | HALLMARK=isolated lymphocytosis, w/ leukocytosis of > 20,000 cells/mL; peripheral smear shows increased mature small lymphocytes and SMUDGE CELLS ARE PATHOGNOMONIC; bone marrow is hypercellular and w/ a left shift |
| Treatment of CLL? | usually palliative once disease is symptomatic |
| myeloproliferative disorder; presents in young to middle-aged adults (median age 55 y.o.); occurs in 3 phases (chronic, accelerated, acute) but inevitably transforms into acute disease | chronic myelogenous leukemia (CML) |
| Symptoms of CML? | fatigue, anorexia, weight loss, low-grade fever, excessive sweating, abdominal fullness (caused by splenomegaly); may have respiratory distress, blurred vision |
| Laboratory findings associated with CML? | HALLMARK=leukocytosis (median WBC of 150,000 cells/mL); Philadelphia chromosome identified in 95% cases; may show anemia and thrombocytosis; bone marrow hypercellular with left shift |
| Treatment of CML? | STI571 (imitanib mesylate) has replaced former standard therapy - effective during chronic phase(hydroxyurea used for iminitab intolerance); allogeneic bone marrow transplantation may be initial tx and only therapy that is curative! |
| Group of CA's characterized by enlargement of lymphoid tissue, spleen, and liver and presence of Reed-Sternberg cells | Hodgkin's disease |
| Demographics of Hodgkin's disease? | common between ages 15-45, more common in males; EVB can be found in 40-50% cases |
| Typical presentation of Hodgkin's disease? | painless cervical, supraclavicular, and mediastinal LA; pain in affected node after ingestion of alcohol may occur |
| What are the two stages of symptoms and what are they? | Stage A (lack of constitutional sx); Stage B (fever, night sweats, weight loss, pruritis, fatigue) |
| What are the various sub-types of Hodgkin's disease? | nodular sclerosis subtype (more common in women); lymphocyte predominant, mixed cellularity, lymphocyte depletion |
| Laboratory studies to diagnose Hodgkin's and non-Hodgkin's lymphoma? | REED-STERNBERG CELLS CONFIRM DIAGNOSIS. Basic staging includes CT of neck, chest, abdomen, and pelvis, as well as bx of bone marrow. Ann Arbor system used to stage Hodgkin's and non-Hodgkin's |
| Treatment of Hodgkin's lymphoma for patients with low-risk stage IA and IIA disease? | radiation therapy is initial tx choice if low risk; good prognosis |
| Treatment of Hodgkin's lymphoma if higher risk or even those with advanced-stage disease? | combination chemotherapy cures > 50% patients |
| malignancies that arise from lymphocytes, 90% cases dervied from B lymphocytes; peak incidence 20-40 y.o. | non-Hodgkin's lymphoma |
| Most common presentation of non-Hodgkin's lymphoma? | diffuse or isolated, painless, persistent LA; bone marrow involvement is frequent |
| Common extralymphatic sites where non-Hodgkin's lymphoma may present include? | GI tract, skin, bone, bone marrow (Burkitt's lymphoma likely to present w/ abdominal fullness) |
| Fever, night sweats, weight loss, puritus, and fatigue are _____ ________ than with Hodgkin's disease. | less likely (but do occur w/ intermediate and high-grade disease) |
| Laboratory studies to evaluate non-Hodgkin's lymphoma? | biopsy persistent, unexplained, enlarged lymph nodes; CT of abdomen, pelvis, chest (for staging); bone marrow bx |
| Treatment of non-Hodgkin's lymphoma? | spontaneous remission may occur; aggressive lymphomas may require aggressive combo chemo or autologous stem cell transplantation |
| single LNR or single ELS | stage I hodgkins: ann arbor criteria |
| two or more LNRs on same side of diaphragm or one solitary ELS and one or more LNRs on same side of diaphragm | stage II hodgkins: ann arbor criteria |
| LNR on both sides of diaphragm; w/ spleen involvement or solitary involvement of an ELS or both | stage III hodgkins: ann arbor criteria |
| diffuse involvement of ELS w/ or w/o node involvement | stage IV hodgkins: ann arbor criteria |
| malignancy of plasma cells, possibly caused by a herpes virus | multiple myeloma |
| replacement of bone marrow leads to failure; bone destruction leads to pain, osteoporosis, lytic lesions, and pathologic fractures; plasmacytomas may cause spinal cord compression | multiple myeloma |
| Most common presentation of multiple myeloma? | median age of dx=65 y.o.; anemia, bone pain (low back, ribs), infection (more prone to recurrent infxn) |
| Less common presentation of multiple myeloma? | renal failure, spinal cord compression, hyperviscosity syndrome; some cases found thru abnormal labs --> hypercalcemia, proteinuria, elevated ESR; f/u electrophoresis abnormal |
| Laboratory studies with multiple myeloma? | anemia (rouleau formation common), lytic lesions or osteoporosis on radiography of axial skeleton (bone scan NOT helpful) |
| Hallmark of multiple myeloma? | monoclonal spike (M-spike) on serum electrophoresis |
| Treatment of multiple myeloma? | options changing rapidly and include thalidomide derivative; bisphosphonates are important adjuncts |
| Initial assessment of a patient with a suspected bleeding disorder should include what? | platelet count, peripheral smear, bleeding time (PT/PTT/INR), thrombin clotting time measures the rate of conversion of fibrinogen to fibrin in the presence of thrombin |
| abnormal decrease in the # of platelets in the blood; most common cause of abnormal bleeding! | thrombocytopenia |
| Causes of thrombocytopenia? | impaired production, increased destruction, splenic sequestration, or dilution |
| self-limited autoimmune (IgG) disorder found most commonly in children of both sexes and is associated with a preceding viral URI | idiopathic thrombocytopenic purpura (ITP) |
| d/o of bleeding that may occur at any age and is more common in women; often coexists w/ other autoimmune diseases | chronic ITP |
| Clinical features of acute ITP? | abrupt appearance of petechiae, purpura, and hemorrhagic bullae on the skin and mucous membranes; possibly epistaxis, oral bleeding, menorrhagia |
| Clinical features of chronic ITP? | develop petechiae on skin and mucous membranes; may also have epistaxis, oral bleeding, and menorrhagia |
| What drug most commonly causes an ITP-like reaction in hospitalized patients? | heparin!! other causes may include sulfonamides, thiazides, cimetidine, gold |
| What are other causes of secondary thrombocytopenia? | SLE and CLL |
| Laboratory findings associated with acute ITP? | decreased platelets (10,000-20,000), eosinophilia, mild lymphocytosis |
| Laboratory findings associated with chronic ITP? | platelet count of 25,000-75,000 |
| Treatment of acute ITP? | usually resolves spontaneously; some patients require steroids or splenectomy |
| Treatment of chronic ITP? | initial tx=high-dose prednisone; splenectomy is definitive and often required; may need platelet transfusion |
| rare but often fatal disorder; found in previously healthy people (20-50 y.o.); characterized by severe thrombocytopenia with purpura, petechiae, pallor, abdominal pain, microangiopathic hemolytic anemia, fever, abnml neuro signs, renal failure | thrombotic thrombocytopenic purpura (TTP) |
| What may precipitate TTP? More common in women or men? | estrogen use, pregnancy, drugs such as quinine and ticlopidine; more common in women |
| disorder similar to TTP but found primarily in children and does not include neurological symptoms | hemolytic-uremic syndrome |
| disorder that causes generalized hemorrhage in pt's w/ severe underlying systemic illness, such as sepsis, tissue injury, obstetric complications, CA, and in severe transfusion reactions | DIC |
| Laboratory findings associated with TTP and HUS? | anemia, red cell fragmentation, NL leukocytes, reticulocytosis, thrombocytopenia, polychromatophilia; ADAMTS 13 (metalloprotease enzyme) is low; LDH markedly elevated; indirect bili increases |
| Treatment of TTP? | large-volume plasmapheresis; prednisone and antiplatelet agents also ahve been used |
| What are the most common causes of acquired platelet dysfunction? | ASA and other NSAIDS |
| What clinical findings may be seen in pt's on ASA and other NSAIDS? | prolonged bleeding time, skin and mucosal bleeding; labs indicate nl number of platelets, but platelet function study results are abnml |
| autosomal dominant, congenital bleeding disorder; most common congenital coagulopathy | von Willebrand's disease |
| reduced levels of factor VIII antigen or ristocetin cofactor | von Willebrand's disease |
| How many types of von Willebrand's disease exist? | 6 major types, all characterized by deficient or defective vWF; Type I accounts for 75-80% cases |
| Clinical features of von Willebrand's disease? | bleeding occurs in nasal, sinus, vaginal, and GI mucous membranes; spontaneous hemarthrosis and soft-tissue bleeds uncommon but can occur |
| What exacerbates bleeding in von Willebrand's disease? What decreases bleeding? | ASA exacerbates it; pregnancy or use of estrogen decreases it |
| Lab findings in von Willebrands? | bleeding time usually prolonged, vWF is low |
| Treatment of von Willebrand disease? | varies according to type of disease; desmopressin acetate useful in type I; Factor VIII concentrates preferred if factor replacement necessary |
| x-linked recessive disease (affecting males) characterized by excessively prolonged coagulation; most severe bleeding disorder and 2nd most common congenital coagulopathy | hemophilia A (factor VIII deficiency or classic hemophilia) |
| Other general characteristics of hemophilia A? | many pt's seropositive for HIV b/c of infected factor VIII transfusions; recent genetic mutation causes 1/3 of all cases of hemophilia A |
| Clinical features of patients severely affected by hemophilia A? | repeated spontaneous hemorrhagic episodes w/ hemarthroses, epistaxis, intracranial bleeding, hematemesis, melena, microscopic hematuria, bleedings into soft tissue and gingiva |
| Clinical features of patients mildly affected by hemophilia A? | excessive bleeding following trauma or surgery |
| Laboratory findings associated with hemophilia A? | PTT is prolonged, factor assay shows reduced factor VIII:C levels (vWF NL) |
| Standard treatment of hemophilia A? | infusion of heat-treated or recombinant factor VIII concentrates; desmopressin may also help elevated factor VIII; AVOID ASA! |
| x-linked recessive d/o (affecting males) similar to hemophilia A but occurs less frequently | factor IX deficiency, Christmas disease |
| mild, autosomal recessive d/o seen primarily in Ashkenazi Jews | factor XI deficiency (rare hemophilia C) |
| Tx of factor XI deficiency? | fresh-frozen plasma |
| Clinical features of factor XI deficiency? | little spontaneous bleeding, but may hemorrhage with surgical procedures and ppx is required |
| Congenital d/o's associated with thrombotic states typically are _______ _______. | autosomal dominant |
| Acquired hypercoaguable states are associated with? | malignancy, pregnancy, OCP's, nephrotic syndrome, immobilization, UC and Crohn's, myeloproliferative disease, anticardiolipin antibodies, DIC, TTP-HUS, antiphospholipid syndrome |
| Congenital causes of hypercoaguable states include? | antithrombin III deficiency, factor V Leiden, protein C deficiency, protein S deficiency, abnormal plasminogen, dysfibrogenemia |
Created by:
rad548