Lymphoma-and-CLL Flashcards
ABVD
doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) is standard;
Rx: Hodgkin’s Stage I and II
- Stages I and II: ABVD followed by radiation. - Some cases may be treated with chemotherapy alone. - Stages III and IV: full-course ABVD followed by Radiation may be added afterward to areas of bulky tumor. Combination therapy with doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) is standard; full-course is six to eight cycles
Complication of Anthracyclines
Late cardiomyopathy
CML vs AML
- AML: acute onset
- AML: blast cells
- CML: splenomegaly
Anemia: Iron deficiency:
why is it common in dialysis patients?
Blood loss during procedures and labs
AL amyloidosis vs AA amyloidosis
- AL amyloidosis: monoclonal gammopathy caused
- AA amyloidosis: chronic inflammatory conditions such as RA or Hodgkin’s
Amyloidosis is a generic term that refers to the extracellular tissue deposition of fibrils composed of low molecular weight subunits of a variety of proteins, many of which circulate as constituents of plasma. These subunit proteins are derived, in turn, from soluble precursors which undergo conformational changes that lead to the adoption of a predominantly antiparallel beta-pleated sheet configuration. At least 30 different human protein precursors of amyloid fibrils are known (table 1). (See ‘Pathogenesis’ above.)
●The two major forms of amyloidosis are the AL (primary) and AA (secondary) types. AL amyloid, the most common form in developed countries, is due to deposition of protein derived from immunoglobulin light chain fragments. It is a plasma cell dyscrasia in which a monoclonal immunoglobulin is detectable in the serum and/or monoclonal light chains in the urine in approximately 80 percent of cases. (See ‘AL amyloidosis’ above and ‘Clinical manifestations’ above.)
AA amyloidosis, the most common form in developing countries, may complicate chronic diseases in which there is ongoing or recurring inflammation, such as chronic infections; rheumatoid arthritis (RA), spondyloarthropathy, or inflammatory bowel disease; or periodic fever syndromes.
Vitamin K dependent clotting factors
II, VII, IX, X
Hemophilia A: The INR is _
normal
measures the time it takes plasma to clot when exposed to tissue factor, which assesses the extrinsic and common pathways of coagulation
PT
Common causes of a prolonged PT include anticoagulants, vitamin K deficiency, liver disease, and disseminated intravascular coagulation (DIC). Some coagulation factor deficiencies may also prolong the PT (table 1).
The international normalized ratio (INR) was developed to allow patients receiving warfarin at steady state to compare values obtained at different times and from different laboratories; it is also commonly used as a surrogate for the PT in bleeding patients and to assess end-stage liver disease as part of the model for end-stage liver diseases (MELD) score. (
Coagulation tests: measures the time it takes plasma to clot when exposed to substances that activate the contact factors coagulation, which assesses the intrinsic and common pathways of coagulation.
aPTT
The activated partial thromboplastin time (aPTT) measures the time it takes plasma to clot when exposed to substances that activate the contact factors coagulation, which assesses the intrinsic and common pathways of coagulation.
Causes of a prolonged aPTT include anticoagulants; liver disease; DIC; von Willebrand disease (VWD); inherited deficiency of factor VIII (hemophilia A), factor IX (hemophilia B), factor XI, or other coagulation factors; acquired factor inhibitors; and antiphospholipid (aPL) antibodies. (See ‘Activated partial thromboplastin time (aPTT)’ above.)
ESLD: bleeding + low fibrinogen: Rx
Cryoprecipitate
Why not FFP? Cryoprecipitate is rich in fibrinogen
Bleeding: Renal failure: Immediate Rx
Meta: ImmediateRx
Desmopressin
Uremia causes platelet dysfunction: desmopressin rapidly/transiently increasing Factor VIII (decreasing aPTT) and VWF factor (decreasing BT).
Low platelets 1 hour after transfusion:
Check HLA compatibility
Bleeding: mixing studies indications
Mixing studies are appropriate in a patient with an unexplained prolongation of a clotting test; they distinguish between an abnormally prolonged clotting time due to a factor deficiency versus a factor inhibitor (typically, an autoantibody).
VWF disease: how can it lead to Factor VIII deficiency?
VWF deficiency leads to increased clearance of Factor VIII
Factor VIII inhibitor: actively bleeding patient:
Rationale for giving Factor VIIa
It bypasses the Factor VIII inhibitor
Bleeding: how are acquired antibodies to coagulation factors detected?
Acquired antibodies to the clotting factors are often diagnosed by mixing studies, in which addition of patient plasma to normal plasma causes the relevant clotting test to become abnormal due to the antibody.
For patients with a low titer factor VIII inhibitor (eg, <5 Bethesda units) and active bleeding, we suggest initial control of active bleeding using a human factor VIII product (recombinant factor VIII or a factor VIII concentrate) rather than another product (Grade 2C). A typical dose is 20 international units/kg intravenously for each Bethesda unit of the inhibitor, plus an additional 40 international units/kg, with monitoring of factor VIII activity 10 minutes following bolus injection, and repeat intravenous bolus dosing if the incremental recovery is not adequate.
Bleeding: Factor VIIIa inhibitor: Rx
Comment: Never seen a case
- low titer: factor VIII
- high titer: Factor VIIa
- Prednisone
For patients with a low titer factor VIII inhibitor (eg, <5 Bethesda units) and active bleeding, we suggest initial control of active bleeding using a human factor VIII product (recombinant factor VIII or a factor VIII concentrate) rather than another product (Grade 2C). A typical dose is 20 international units/kg intravenously for each Bethesda unit of the inhibitor, plus an additional 40 international units/kg, with monitoring of factor VIII activity 10 minutes following bolus injection, and repeat intravenous bolus dosing if the incremental recovery is not adequate. (See ‘Control of active bleeding’ above.)
●For patients with a high titer factor VIII inhibitor (eg, ≥5 Bethesda units) and active bleeding, we suggest using an activated prothrombin complex concentrate (aPCC; eg, factor VIII inhibitor bypassing activity [FEIBA]) or recombinant human factor VIIa (rfVIIa) rather than a human factor VIII product (Grade 2C). Human factor VIII generally cannot be given in high enough amounts to overcome the inhibitor. Recombinant porcine factor VIIIa is also an option. The choice of product depends on availability, initial or previous responses, and clinician preference. (See ‘Control of active bleeding’ above.)
●To eliminate factor VIII inhibitors we recommend the use of prednisone at an initial oral dose of 1 mg/kg per day in all patients (Grade 1B). Based upon weak evidence from the literature and our own experience, we suggest that oral cyclophosphamide (2 mg/kg per day) be added to the initial prednisone treatment regimen (Grade 2C). (See ‘Eliminating the inhibitor’ above.)
●Inhibitors of prothrombin are frequently associated with antiphospholipid antibodies. Treatment of active bleeding is done with fresh frozen plasma (FFP). Modalities associated with factor VIII antibodies may also be useful. (See ‘Prothrombin (factor II) inhibitors’ above and ‘Treatment of factor VIII inhibitors’ above.)
PBS: polychromasia: Implies
Meta: implies, signifies
Indicating an increase in young RBCs
RBC Description terms
- Anisocytosis: Differently sized RBCs
- Poikilocytosis: Differently shaped RBCs
- Polychromasia: Indicating an increase in young RBCs
- Microcytosis: Small RBCs
- Macrocytosis: Large RBCs
- Schistocytes: Fragmented RBC forms
PBS: RBC forms
A, Schistocytes. B, Macrocytes. C, Spherocytes. D, Teardrops, bizarre shapes.
Hypochromic, microcytic: Ddx
Iron deficiency
Thalassemia
Anemia of chronic disease
PBS: describe and Dx
Burr cell
Uremia
PBS: describe and dx
Acanthocyte
Liver disease
Abetalipoproteinemia
Echinocytes — Echinocytes (burr cells) are red cells with serrated edges over the entire surface of the cell, and often appear crenated in a blood smear (picture 3). The projections of the red cell membrane are smaller and much more uniform in shape and distribution in echinocytes than in acanthocytes.
Acanthocytes — Acanthocytes have only a few spicules of varying size that project from the red cell surface at irregular intervals (picture 4). Spur cells appear to be the extreme form of acanthocytes and are seen in patients with severe liver disease.
Causes
●Echinocytes are often found as artifacts on blood smears and have been demonstrated in both end-stage renal disease and in liver disease.
●Patients with liver disease often have acanthocytes, echinocytes, and target cells on the peripheral blood smear. Spur cells are the extreme form of acanthocytes. They are probably acanthocytes that have been additionally remodeled by an enlarged spleen and considerably enriched in cholesterol. (See ‘Pathophysiology’ above.)
PBS: schistiocyte: DDx
Microangiopathic hemolytic anemia (e.g., TTP, DIC)
Malignant hypertension
Prosthetic heart valve
PBS: spherocytes: Ddx
Autoimmune hemolytic anemia
Hereditary spherocytosis
Macrocytes: Ddx
Vitamin B12 deficiency
Folate deficiency
Myelodysplastic syndrome
Liver disease
Hypothyroidism
PBS: Teardrops, nucleated RBCs, bizarre forms: Ddx
Myelofibrosis Marrow infiltration (e.g., tumor, tuberculosis)
PBS: Bite cells: Ddx
G6PD, unstable hemoglobinopathy
PBS: Leukopenia with hyposegmented PMNs
Myelodysplastic syndrome
Stress, infection
Pelger-Huët anomalyBone marrow examination with cytogenetics/FISH if myelodysplastic syndrome suspected
