H/O Flashcards
(112 cards)
Thrombotic thrombocytopenic purpura
a clinical diagnosis that requires the presence of thrombocytopenia and microangiopathic hemolytic anemia, which is confirmed by schistocytes on the peripheral blood smear. Patients may also have fever; kidney manifestations such as hematuria, elevated creatinine level, and proteinuria; and fluctuating neurologic manifestations, but the absence of these symptoms does not exclude the diagnosis. Assays for ADAMTS-13 activity and inhibitor titer are available but are best used for prognosis rather than to guide therapy, because TTP requires immediate treatment with plasma exchange that cannot be delayed until laboratory test results are available.
Major bleeding associated with vitamin K antagonists
Should be treated by reversing anticoagulation with 4-factor prothrombin complex concentrate in addition to intravenous vitamin K.
4-factor prothrombin complex concentrate
Contains all four vitamin K–dependent coagulation factors (factors II, VII, IX, and X) Unlike fresh frozen plasma (FFP), 4f-PCC is stored at room temperature, does not require ABO typing, and can be infused quickly because of its small volume, thus reducing the time to delivery of therapy. Compared with FFP, 4f-PCC has been shown to more rapidly achieve hemostasis in patients with visible or musculoskeletal bleeding with less risk of fluid overload and no difference in thromboembolic events. This agent has therefore been approved by the FDA for urgent reversal of coagulation factor deficiencies related to vitamin K antagonist therapy for adult patients with acute major bleeding, as well as for adult patients in need of urgent surgery or an invasive procedure.
chronic thromboembolic pulmonary hypertension (CTEPH)
efined as a mean pulmonary artery pressure of greater than 25 mm Hg, with normal pulmonary capillary wedge pressure, left atrial pressure, and left ventricular end-diastolic pressure. It typically occurs within 2 years following a pulmonary embolism (PE), affecting 3.8% of patients, although only about 50% of these have a history of clinically detected PE If the V/Q scan suggests CTEPH, confirmatory right heart catheterization with pulmonary artery pressure measurements and pulmonary arteriography is indicated.
paroxysmal nocturnal hemoglobinuria (PNH)
Hemolytic anemia, pancytopenia, or unprovoked atypical thrombosis. Hemolysis is caused by the absence of decay-accelerating factor (CD55) and the membrane inhibitor of reactive lysis (CD59), which are glycosylphosphatidylinositol-dependent complement regulatory proteins Mutations in the PIG-A gene lead to the reduction or absence of glycosylphosphatidylinositol, an important erythrocyte-anchoring protein
Budd-Chiari syndrome associated with
Half of patients with idiopathic BCS had an acquired mutation in JAK2, without overt suggestion of a myeloproliferative neoplasm. Therefore, testing for JAK2 is part of the diagnostic testing protocol that includes consideration of paroxysmal nocturnal hemoglobinuria in the differential diagnosis of splanchnic vein thrombosis.
Hemophilia
Hemophilia A results from factor VIII deficiency and hemophilia B from factor IX deficiency; both produce a prolongation of the activated partial thromboplastin time that fully corrects in a mixing study.
hypereosinophilic syndrome (HES)
An elevated eosinophil count (>1500/µL [1.5 × 109/L]) without a secondary cause and evidence of organ involvement are diagnostic. Causes of eosinophilia are described in the CHINA: connective tissue diseases, helminthic infection, idiopathic [HES], neoplasia, allergy
Urticaria pigmentosa
Pruritic yellow to red or brown macules, papules, plaques, and nodules. Systemic mastocytosis with eosinophilia is characterized by urticaria pigmentosa. The most common noncutaneous findings are gastrointestinal and include symptoms such as abdominal pain, diarrhea, nausea, and vomiting.
identification of an inherited thrombophilia
Often does not change treatment decisions in a patient with VTE (does not reliably predict risk of recurrence or influence duration of recommended anticoagulation), evidence-based guidelines recommend against routine thrombophilia testing. In patients with an unprovoked proximal DVT, the recommendation for long-term anticoagulation would not be altered by the results of such testing, thus, it would not be helpful. Testing may be indicated, however, in patients with VTE at intermediate risk for recurrence by traditional predictors in whom finding a strong thrombophilic risk might alter therapeutic decisions.
transfusing select patients who are immunocompromised to reduce the risk of transfusion-associated graft-versus-host disease and febrile nonhemolytic transfusion reaction.
Leukoreduced and irradiated erythrocytes should be used (those with severe, inherited T-cell immunodeficiency syndromes or Hodgkin lymphoma or recipients of allogeneic or autologous hematopoietic stem cell transplantation, purine analog–based chemotherapy [fludarabine, cladribine, deoxycoformycin], alemtuzumab, or rabbit antithymocyte globulin therapy) are at increased risk of developing transfusion-associated graft-versus-host disease (ta-GVHD)
Washed erythrocytes
considered for patients with a history of severe allergic reactions to transfusions patients who are IgA deficient
acute lymphoblastic leukemia (ALL) in older patients
Diagnosis: presence of 25% or more lymphoblasts on bone marrow examination. Cytochemical stains and flow cytometry can help distinguish ALL from acute myeloid leukemia (AML) and B-cell from T-cell ALL prognosis for an older patient with ALL has traditionally been poor, with Philadelphia chromosome [t(9;22)] positivity indicating worse outcomes The most significant advance in the treatment of older patients with Philadelphia chromosome–positive disease is TKI therapy. The results of dasatinib and dexamethasone therapy are better than those for traditional chemotherapy, with less toxicity. For older patients who have Philadelphia chromosome–negative ALL, no clear standard cytotoxic chemotherapy regimen exists. However, TKI therapy can provide disease control for greater than 1 year with much less toxicity. Combination regimens such as hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (Hyper-CVAD) can cure adults with ALL, but are too toxic for use in elderly populations. The paradox of ALL in older adults is that although less aggressive regimens are less toxic, they compromise the ability to control the leukemia.
HLA-matched platelets.
Patients experiencing platelet transfusion refractoriness because of alloimmunization should receive
β-thalassemia
Hemolytic anemia, microcytosis, and target cells are typical of β-thalassemia, which is associated with slightly increased hemoglobin A2 and some residual hemoglobin F.
elevated erythropoietin level and hypoxemia
secondary erythrocytosis. Don’t need bone marrow biopsy.
acquired hemophilia
associated with high titers of inhibitor. Recombinant activated factor VII is used to treat the bleeding episodes. Patients with low titers of inhibitor (measured in Bethesda units) may be treated with factor VIII concentrates. Patients with high inhibitor titers (>5 Bethesda units) require treatment with recombinant factor VIIa or prothrombin complex concentrates designed to activate factor X and secure hemostasis independent of factor VIII and the intrinsic pathway. Patients may require immunosuppression for inhibitor eradication.
In some patients with fluctuating INRs while taking warfarin
daily supplementation with low-dose vitamin K (100-150 µg/d) can stabilize the INR.
parvovirus and sickle cell disease
Parvovirus B19 infection can cause acquired pure red cell aplasia in an otherwise functionally asplenic patient with sickle cell disease.
immune thrombocytopenic purpura treatment
without evidence of bleeding and platelet counts greater than 30,000 to 40,000/µL (30-40 × 109/L) have less than a 15% chance of developing more severe thrombocytopenia requiring treatment and can be managed with careful observation. Repeat the complete blood count at a designated interval, generally 1 to 2 weeks, u ITP is a diagnosis of exclusion, supportive clinical findings include an otherwise normal blood count and the absence of additional organ dysfunction. Platelets on the peripheral blood smear are large because they typically have been recently released from the marrow, and the enhanced hemostatic function of these young platelets may account for less severe bleeding symptoms than those associated with other diseases with a similar platelet count. therapy may be required for patients with platelet counts lower than 30,000 to 40,000/µL (30-40 × 109/L) or with bleeding. Initial therapy consists of glucocorticoids. Patients who do not respond to glucocorticoid therapy should be treated with an additional agent such as intravenous immune globulin or anti-D immune globulin or rituximab. Splenectomy leads to a sustained remission in 75% of patients.
Isolated superficial venous thrombophlebitis
Duplex ultrasonography is indicated to assess for the possibility of an associated deep venous thrombosis (DVT) in patients with isolated superficial venous thrombophlebitis (SVT), because DVT or pulmonary embolism risk increases in patients with SVT of the great or small saphenous vein, with extremity swelling more pronounced than would be expected from the SVT alone, and with progressive symptoms. Nonextensive SVT, defined as less than 5 cm in length and not near the deep venous system, may be treated with only symptomatic therapy consisting of analgesics, anti-inflammatory medications, and warm or cold compresses for symptom relief, because the risk of progression into the deep venous system and of PE is low.
folate deficiency
Those with generalized malnutrition or poor nutrition, can become folate deficient in weeks to months because of relatively limited stores of folate in the body. Measuring serum folate levels is typically unreliable in diagnosing folate deficiency, because folate levels increase rapidly after a single folate-containing meal. Plasma homocysteine levels increase in folate deficiency, whereas homocysteine and methylmalonic acid levels are increased in cobalamin deficiency.
Treatment of essential thrombocythemia
Hydroxyurea plus low-dose aspirin is the best treatment option for essential thrombocythemia when treatment is required in patients older than 60 years, those with a platelet count greater than 1 million/µL (1000 × 109/L), or those with a history of thrombosis.
Heyde’s syndrome
a syndrome of gastrointestinal bleeding from angiodysplasia in the presence of aortic stenosis Caused by the induction of Von Willebrand disease type IIA (vWD-2A) by a depletion of Von Willebrand factor (vWF) in blood flowing through the narrowed valvular stenosis. Can also get a MAHA from valvular replacement/disease.




