Anemia Flashcards
_____ means a low mean corpuscular volume (MCV) <80
Microcytic anemia
Microcytic anemia causes
These are most commonly a result of iron deficiency, anemia of chronic disease, thalassemia, sideroblastosis, and lead poisoning
_____ is characterized by an elevated MCV >100.
Macrocytic anemia
MCC of Macrocytic anemia
This is most commonly
from vitamin B12 or folic acid deficiency but can also result from the toxic effects of
alcohol, liver disease, or chemotherapeutic agents such as methotrexate or medications
such as zidovudine (AZT) or phenytoin
Packed RBCs are used to maintain a hematocrit______
> 25–30%.
Normocytic anemia, Reticulocyte Count <3%
(bad bone marrow response) ddx
Early stages iron deficiency anemia
• Early stages anemia of chronic disease
• Aplastic anemia
• Chronic renal failure
Iron absorption is tightly regulated. A man requires about ______ per day and a woman about ____mg per day on average.
1 mg
2–3
As the hematocrit approaches 30%, symptoms _______
of fatigue and poor exercise tolerance may develop.
As the hematocrit lowers to 25%, ______
tachycardia, palpitations, dyspnea on exertion, and pallor develop
A_____may develop in any patient with
moderately severe anemia
systolic ejection murmur (“flow” murmur)
Symptoms specific to iron deficiency are rare and cannot be relied upon to determine the
diagnosis. These include ______
brittle nails, spoon shaped nails, glossitis, and pica.
A______ is the most characteristic finding of iron deficiency anemia
low serum ferritin <10 ng/mL
Low ferritin has good specificity ___but poor sensitivity ____
(>99%)
(60%);
The serum iron is____ and the total iron binding capacity is ____. The RDW is elevated.
low
high
A defect in the ability to make use of iron sequestered in stores within the reticuloendothelial system. It can be either microcytic or normocytic
Anemia of Chronic Disease
____ a regulator of iron metabolism, plays an important role in anemia of
chronic disease
Hepcidin,
In states where hepcidin level is abnormally high (e.g., inflammation), serum
iron falls due to ____
iron trapping within macrophages and liver cells and decreased gut iron absorption.
Hepcidin inhibits iron transport by binding to the iron export channel _____
located on the surface of gut enterocytes and the plasma membrane of macrophages
ferroportin
In genetic diseases where hepcidin level is abnormally low, what are the CX?
iron overload may occur (hemochromatosis) due to unwarranted ferroportin facilitated iron influx.
Dx of Anemia of chronic DSE
The serum ferritin level is normal or elevated. The serum iron level and total iron binding capacity (TIBC) are both low. The reticulocyte count is low
Anemia of chronic DSE TX
Iron supplementation and erythropoietin will not help, except in ______
renal disease and anemia caused by chemotherapy or radiation therapy
A microcytic anemia caused by a disorder in the synthesis of hemoglobin characterized
by trapped iron in the mitochondria of nucleated RBCs
Sideroblastic Anemia
Sideroblastic Anemia
The hereditary form is from either a
defect in_____ or ____
aminolevulinic acid synthase or an abnormality in vitamin B6 metabolism
Sideroblastic Anemia
Acquired forms are from drugs such as ______. Lead poisoning can cause
sideroblastic anemia as well.
chloramphenicol, isoniazid, or alcohol
Sideroblastic anemia may progress to _____in a small percentage of patients
acute myelogenous leukemia
Dx of sideroblastic anemia
The serum ferritin level is elevated.
Transferrin saturation is very high, and therefore the TIBC is very low. The serum iron level is high
Sideroblastic anemia
The most specific test is a _____
Prussian Blue stain of RBCs in the marrow that will reveal the ringed sideroblasts
Sideroblastic anemia is the only microcytic anemia in which _____
serum iron is elevated.
Sideroblastic anemia Tx
Some patients, especially those with
INH-associated sideroblastic anemia, will respond to______
pyridoxine therapy 2-4 mg per day
The hereditary underproduction of either the alpha or beta globin chains of the
hemoglobin molecule resulting in a hypochromic, microcytic anemia
Thalassemia
Alpha thalassemia is more common in____
Beta thalassemia is more common in ___
Asian populations.
Mediterranean populations
In alpha thalassemia, 1 gene deleted yields a normal patient. What are the CBC, Hb and MCV levels?
The CBC is normal, the
hemoglobin level is normal, and the MCV is normal.
Alpha thalassemia
Individuals with 2 genes deleted have a mild anemia with hematocrits ranging from _____ with a strikingly____
30–40%
low MCV.
Alpha thalassemia
Those with 3 genes deleted have more profound anemia with hematocrits _____ as
well as the very low ____. Four-gene-deleted alpha thalassemia patients die in utero
secondary to gamma chain tetrads called ______
22–32%
MCV
hemoglobin Barts.
These patients with beta thalassemia major,
also known as Cooley anemia, become severely symptomatic starting at 6 months because?
the age when the body would normally switch from fetal hemoglobin to adult hemoglobin
Cx of beta thalassemia major,
They are later symptomatic from hemochromatosis, cirrhosis, and CHF from chronic anemia and transfusion dependence.
Clues to the diagnosis of thalassemia trait is a ________
mild anemia with a profound
microcytosis
_____differentiates which type of thalassemia is present.
Hemoglobin electrophoresis
Hb electrophoresis
In beta thalassemia, there is an increased level of_______
hemoglobin F and hemoglobin A2.
Those with alpha thalassemia will have normal amounts of hemoglobins F and A2. Tetrads of beta chains are called ___
hemoglobin H.
Hemoglobin H is present in _____
alpha thalassemia with 3 of 4 genes
deleted.
____ are present in all forms of thalassemia trait and thalassemia major.
Target cells
Thalassemia traits of both the alpha and beta types do not require specific treatment.
Beta thalassemia major patients require____
blood transfusions once or twice a month
Thalassemia
The chronic transfusions lead to iron overload, which requires treatment with _______
deferasirox. Oral deferasirox is the standard of care.
The most common cause of B12 deficiency is ______ which is a disorder resulting in decreased intrinsic factor production due to autoimmune destruction of parietal cells.
pernicious anemia,
Various forms of malabsorption such as ____, _____, _____ can block absorption of vitamin B12.
sprue, regional enteritis, and blind loop syndrome
DX of Anemia from B12 def
The WBCs have ______
The red cells are characterized by____
hypersegmented neutrophils with a mean lobe count >4.
macro-ovalocytes
B12 and folate deficiency produce ____. The hematologic pattern of vitamin B12 deficiency
is indistinguishable from folate deficiency.
oval macrocytes
The most specific test is a____.
_____confirm the etiology as pernicious anemia.
low B12 level
Antibodies to intrinsic factor and parietal cells
An elevated ____ occurs with B12
deficiency and is useful if the B12 level is equivocal
methylmalonic acid level
Tx of Vit B12 def
Parenteral route is recommended for patients with_____. IV dosing is not recommended because that would result in most of
the vitamin being lost in the urine.
neurologic manifestations
of B12 deficiency
Response of vitamin B12 deficiency anemia to treatment is usually rapid, with reticulocytosis
occurring within_____
2–5 days and hematocrit normalizing within weeks
Vit B12 def
Treatment with cobalamin
effectively halts progression of the deficiency process but __
might not fully reverse more
advanced neurologic effects.
Patients who have vitamin B12 deficiency with associated megaloblastic anemia might experience severe_____and fluid overload early in treatment due to increased erythropoiesis, cellular uptake of potassium, and increased blood volume
hypokalemia
____can correct the hematologic abnormalities of B12 deficiency, but not
the neurologic abnormalities
Folic acid replacement
Folic acid def
Occasionally, increased requirements from pregnancy, skin loss in diseases like ___
eczema, or increased loss from dialysis and certain anticonvulsants such as phenytoin may occur
Hemolytic anemia
Hereditary Membrane problems
heriditary spherocytosis, hereditary elliptocytosis
Hemolytic anemia
Hereditary Metabolism: problems
G6PD deficiency, pyruvate
kinase deficiency
Hemolytic anemia
Hereditary Hemoglobin:
genetic abnormalities (Hb S, Hb C, unstable)
Hemolytic anemia, acquired
- Autoimmune: ______
- Alloimmune: ______
- Drug-associated
warm antibody type, cold antibody type
hemolytic transfusion reactions, hemolytic disease of the newborn, allografts (especially stem cell transplantation)
_____may occur if the intravascular hemolysis is particularly rapid.
Fever, chills, chest pain, tachycardia, and
backache
Patients with hemolytic anemias generally have a normal MCV, but the MCV may
be slightly elevated because _____
reticulocytes are somewhat larger than older cells.
In HA
There should not be bilirubin in the urine because ____
indirect bilirubin is bound to
albumin and should not filter through the glomerulus
Patients with chronic hemolytic anemia need to be maintained on _____ as there is an increase in cell turnover.
chronic folic acid therapy,
A hereditary form of chronic hemolysis ranging from asymptomatic to severe,
overwhelming crisis. It is characterized by irreversibly sickled cells and recurrent painful crises
Sickle Cell Disease
____is due to a substitution of a valine for glutamic acid as the sixth amino acid of the beta globin chain
Hemoglobin S
A sickle cell acute painful crisis can be precipitated by______
hypoxia, dehydration, acidosis, infection, and fever. However, the crisis may occur without the presence of these factors
Sickle cell crisis is usually not associated with an _____
increase in hemolysis or drop in hematocrit.
Causes of hemolysis in pts with sickle cell crisis
When increased hemolysis occurs, another etiology such as concomitant glucose
6 phosphate dehydrogenase deficiency (G6PD) or acute splenic sequestration in a child should be considered.
A sudden drop in hematocrit may also be caused by Parvovirus B19 infection or folate deficiency
SSx of sickle cell crisis
Chronic manifestations include renal concentrating defects (isosthenuria),
hematuria, ulcerations of the skin of the legs, bilirubin gallstones, aseptic necrosis
of the femoral head, osteomyelitis, retinopathy, recurrent infections from Pneumococcus or Haemophilus, growth retardation, and splenomegaly followed in adulthood by autosplenectomy
Severe manifestations of sickle cell crisis
acute chest syndrome consisting of severe chest pain, fever, leukocytosis, hypoxia, and infiltrates on the chest
x-ray.
sickle cell crisis
Priapism can occur from infarction of the _____
prostatic plexus of veins
The ____ is the most specific test for SCD
hemoglobin electrophoresis
The______ is a quick screening test used to diagnose evidence of sickle cell trait and cannot distinguish between trait and homozygous disease.
sickle prep (or Sickledex)
An acute sickle cell pain crisis is treated with ______
fluids, analgesics, and oxygen
Sickle cell crisis
_____ is the preferred agent because
it covers Pneumococcus and Haemophilus influenza.
Ceftriaxone
Sickle cell crisis
_____ is used to decrease the
frequency of the vaso-occlusive pain crisis.
Hydroxyurea
Various forms of acquired hemolytic anemias resulting from the production of
IgG, IgM, or activation of complement C3 against the______
red cell membrane
AIHA
The lysis can be either extravascular or intravascular but is far more often
____
extravascular
Sites of extravascular hemolysis
Destruction of the cells most often occurs
through macrophages in the spleen or by Kupfer cells in the liver
AIHA
The most common drugs are the ____
penicillins, cephalosporins, sulfa drugs, quinidine, alphamethyldopa, procainamide, rifampin, and thiazides
____can also lead to autoimmune hemolytic anemia
Ulcerative colitis
______ is an IgM antibody produced against the red cell in association with malignancies such as lymphoma or Waldenstrom macroglobulinemia and infections such as Mycoplasma or mononucleosis.
Cold agglutinin disease
Cold agglutinin destruction occurs predominantly in the ______
liver
Cold agglutinin disease results in _____
cyanosis of the ears, nose, fingers,
and toes.
AIHA Dx
Autoimmune hemolysis gives a
normocytic anemia, reticulocytosis, increased LDH, absent or decreased haptoglobin, and increased indirect bilirubin, as can all forms of hemolysis
The _____ is the specific test that diagnoses autoimmune, cold agglutinin,
and often even drug-induced hemolysis
Coombs test
AIHA
_____are often present on the smear
Spherocytes
More severe autoimmune hemolysis is treated with______
steroids first.
Cold agglutinin disease is primarily managed with ______
avoiding the cold
Most cases of cold agglutinin disease are mild, but in those who have severe disease despite conservative measures,____ can be used
azathioprine, cyclosporine,
or cyclophosphamide can be used. Rituximab is also useful
_____ and ____ don’t work well with cold agglutinin disease because the destruction
occurs in the liver
Steroids and splenectomy
A chronic mild hemolysis with spherocytes, jaundice, and splenomegaly from a
defect in the red cell membrane
Hereditary Spherocytosis
An autosomal dominant disorder where the loss of _____ in the red cell membrane
results in the formation of the red cell as a sphere, rather than a more flexible and
durable biconcave disc
spectrin
HS
Severe anemia occasionally occurs from ____
folate deficiency or Parvovirus B19 infection such as in sickle cell disease
HS Dx
CBC findings
A normal to slightly decreased MCV anemia with the elevated LDH; indirect bilirubin
and reticulocyte count similar to any kind of hemolysis
Although spherocytes may be
present with autoimmune hemolysis, hereditary spherocytosis has a negative _____
Coombs test
The cells have increased sensitivity to lysis in hypotonic solutions known as an ______
osmotic fragility test
A red cell membrane defect leading to intermittent dark urine and venous thrombosis and a chronic form of hemolysis
Paroxysmal Nocturnal Hemoglobinuria (PNH
PNH
A red cell membrane defect in ____ allows
increased binding of complement to the red cell leading to increased intravascular hemolysis
phosphatidyl inositol glycan A (PIG-A)
It is a clonal stem-cell disorder and therefore can develop into ____ and _____
aplastic anemia and leukemia as
well
PNHA
Thrombosis of major venous structures,
particularly the ______, is a common cause of death in these patients
hepatic vein (Budd-Chiari syndrome)
The hemoglobinuria is most commonly in the first morning urine because the
_____
hemolysis occurs more often when patients develop a mild acidosis at night.
Besides the usual lab findings of hemolysis, such as an increased LDH, bilirubin,
and reticulocyte count, these patients have brisk intravascular hemolysis and therefore have a _______
low haptoglobin and hemoglobin in the urine.
_____ occurs when the capacity
of renal tubular cells to absorb and metabolize the hemoglobin is overwhelmed and sloughed off iron-laden cells are found in the urine
Hemosiderinuria
The gold standard test is______on white and red cells. In PNH, levels are low or absent
flow cytometry for CD55 and CD59
PNH
Some patients with few or no symptoms require ______
only folic acid and possible iron supplementation
PNH
In the anemic patient with signs of hemolysis, ______ is often given to slow the
rate of red blood cell destruction
prednisone
PNH Tx
In the patient with acute thrombosis, ___
thrombolytic therapy (streptokinase, urokinase, or tissue plasminogen activator) is often administered, followed by long-term anticoagulation drugs to help prevent further blood clots
PNH is often associated with _____
bone marrow failure
______has been the mainstay of curative therapy for PNH
Allogeneic bone marrow transplantation
The hereditary deficiency of an enzyme for producing the reducing capacity necessary
for neutralizing oxidant stress to the red cell resulting in acute hemolysis
Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency
Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency
The most common type of oxidant stress is actually from______
infections, not drugs
Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency
The most commonly implicated drugs
are ________
sulfa drugs, primiquine, dapsone, quinidine, and nitrofurantoin
Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Dx
The usual findings of an intravascular hemolysis include ______
high LDH, bilirubin, and
reticulocyte count with a normal MCV, low haptoglobin, and hemoglobinuria
PBS of G6PD
____ are precipitated hemoglobin inclusions seen in red cells.
____are seen on smear indicating
the removal of the Heinz bodies.
Heinz bodies
Bite cells
The definitive test is the _____, which can be falsely normal immediately after an episode of hemolysis. Hence, the level is best tested about 1 week after the event.
G6PD level
_____ is failure of all 3 cell lines produced in the bone marrow, resulting in anemia, leukopenia, and thrombocytopenia (pancytopenia). The marrow is essentially
empty with the absence of precursor cells.
Aplastic anemia
Radiation, toxins such as benzene, drugs such as ______ can all cause aplastic anemia
NSAIDs, choramphenicol, alcohol, and chemotherapeutic alkylating agents
Aplastic anemia can also be caused by infections such as_____
hepatitis, HIV, CMV, Epstein-Barr virus, or Parvovirus B19 in immunocompromised patients.
AA
The most common single etiology is ___
idiopathic
AA Tx
Bone marrow transplantation should be carried out whenever the patient
is young and healthy enough to withstand the procedure and there is a donor available.
Allogeneic transplant can cure up to_____
80–90% of patients under 50.
When a bone marrow transplantation is not possible, ______
immunosuppressive agents should be tried
It is believed that _____ are primarily causal in the bone marrow failure,
T lymphocytes
_____ is the rapid onset of bone marrow failure from the derangement of the pluripotent stem cell, causing the relentless destruction of the normal production of
the entire bone marrow
Acute leukemia
Most cases of acute leukemia arise with no apparent cause, but there are several
well known associations: ______
radiation exposure, benzene, chemotherapeutic agents such as melphalan and etoposide, and some retroviruses.
Genetic disorders such as _____ and ____ can result in an increased incidence of leukemia
Down syndrome and Klinefelter
MC complaint for ALL
Fatigue from anemia is the most common presenting complaint
___ is more common in children and______ is more common in adults, but they are indistinguishable clinically
Acute lymphocytic leukemia (ALL)
acute myelogenous leukemia (AML)
Disseminated intravascular coagulation (DIC) is associated with_____
M3 promyelocytic leukemia
CNS involvement is most characteristic of ______
M4 and M5 monocytic leukemia
Cx of leukostasis
This results from sludging of the leukemic cell in the vasculature, resulting in headache, dyspnea, confusion, and brain
hemorrhage.
A bone marrow biopsy showing _____confirms the diagnosis of acute leukemia
> 20% blasts
_____ is characterized by the presence of Auer rods, myeloperoxidase, and esterase
AML
ALL is characterized by the presence of the ______ and _____
common ALL antigen (CALLA) and terminal deoxynucleotidyl
transferase (TdT
ALL Tx
Inducing a remission means a removal of over _____ of the leukemic cells in the body and the elimination of peripheral blasts in circulation.
99.9%
After chemotherapy, adults with AML or ALL should be referred for ______
allogeneic bone marrow transplantation
The initial chemotherapy for AML is _____ and _____
cytosine arabinoside (AraC) and either daunorubicin or idarubicin
Promyelocytic leukemia is managed with the addition of the ______
vitamin A derivative all-trans-retinoic acid (ATRA).
ALL Tx
ALL patients must also undergo prophylaxis of the central nervous system to prevent relapse there. The best agent for this is _______
intrathecal methotrexate.
A chronic myeloproliferative disorder characterized by the massive overproduction of myeloid cells. These cells retain most of their function until later in the course of the disease
Chronic Myelogenous Leukemia (CML)
Chromosome associated with CML
Philadelphia chromosome
What is the Philadelphia chromosome?
The Philadelphia chromosome is a translocation between chromosomes 9 and 22, resulting in a gene producing an enzyme with tyrosine kinase activity
MC Sx of AML
The most common symptoms are fatigue, night sweats, and low-grade fever
Main feature of AML
The main feature of the disease is an elevated white blood cell count consisting
predominantly of neutrophils with a left shift
_____ is characteristic of CML and all myeloproliferative disorders such as polycythenia vera
Basophilia
The ______ is a far more specific test for CML and should be done in a patient with a markedly elevated white cell count
Philadelphia chromosome
The best initial therapy for CML is ____ which is also known by the manufacturer’s
name, Gleevec
imatinib,
Imatinib is a _____produced by
the Philadelphia chromosome
direct inhibitor of the tyrosine kinase
Bone marrow transplantation is no longer
the clear first choice as therapy for CML. This is because of the _______
extraordinary response to imatinib, as well as the high mortality associated with the bone marrow transplantation itself
Massive overproduction of mature, but still leukemic, lymphocytes usually from
the monoclonal production of B lymphocytes
Chronic Lymphocytic Leukemia (CLL)
Staging of CLL
Stage 0: lymphocytosis alone Stage 1: lymphadenopathy Stage 2: splenomegaly Stage 3: anemia Stage 4: thrombocytopenia
Staging is important because the survival of untreated stage 0 and stage 1 disease is _____ years even without treatment
10–12
CLL can be associated with various autoimmune phenomena such as _____ and ______
thrombocytopenia and autoimmune
hemolytic anemia
CLL is strongly suspected when____
an older patient has a marked elevation in the white cell count with a marked lymphocytic predominance in the range of 80–98% lymphocytes.
______seen on a smear are characteristic of CLL
“Smudge cells”
T or F
Early stage CLL with only an elevated white cell count or enlargement of lymph
nodes is not treated
T
CLL
Those with more advanced-stage disease should receive initial therapy with _____
fludarabine
_____ classified as a subtype of chronic lymphoid leukemia, makes
up approximately 2% of all leukemias
Hairy cell leukemia (HCL),
Etiology of HCL
The malignant B lymphocytes (“hairy cells” ) accumulate in the bone marrow, interfering with the production of normal cells commonly causing pancytopenia
HCL Dx is confirmed by:
The diagnosis can be confirmed by viewing the cells with a special stain known as TRAP (tartrate resistant acid phosphatase).
HCL
Bone marrow failure is caused by the _______ and _____
accumulation of hairy cells and reticulin fibrosis in the bone marrow, as well as by the unfavorable effects of dysregulated cytokine productio
HCL
_____ and ____ are the most common firstline therapies.
Purine analogs cladribine (2CDA) and pentostatin
_____ is an idiopathic disorder that is considered “pre-leukemic,” in that a number of people go on to develop acute myelogenous leukemia (AML)
MDS
MC genetic defect of MDS
5q deletion or “5q–.”
HCL Tx
You may find a bi-lobed neutrophil called a ______ which is characteristic.
Pelger- Huet cell
HCL
Disease-specific therapy consists of the TNF inhibitor_______
lenalidomide or thalidomide.
A disorder of red cell production. Red cells are produced in excessive amounts in
the absence of hypoxia or increased erythropoietin levels
PCV
Why is there pruritus with PCV
Pruritis (approximately 40% of patients), particularly after exposure to warm water
such as in a shower or bath; possibly caused by abnormal histamine or prostaglandin production
PCV
The most specific test is the ___
Janus Kinase or JAK-2
PCV Tx
Phlebotomy is the primary treatment; ______may be used in addition to or
as an alternative. Aspirin is used to reduce the risk of thrombotic events.
hydroxyurea
_____is a type of platelet cancer. Platelet count may be over a million. There is either thrombosis or bleeding
Essential thrombocythemia
Tx of ET
Treat with hydroxyurea and sometimes anagrelide.
A clonal abnormality of plasma cells resulting in their overproduction replacing
the bone marrow as well as the production of large quantities of functionless immunoglobulins.
Multiple Myeloma
Dx of MM
A protein electrophoresis with a markedly elevated monoclonal immunoglobulin spike is present in almost all cases
MC Ig in MM
This is most commonly IgG but may be IgA, IgD, or rarely a combination of two of these.
MC lesions in MM
Most commonly involved are the vertebrae, ribs, pelvic bones, and bones of the thigh and upper arm.
A bone marrow biopsy with ______confirms a diagnosis of multiple myeloma
> 10% plasma cells
MM
_____ is often not detected by a standard protein test on a urinalysis, which mainly is meant to detect albumin
Bence-Jones protein
MM
Younger patients (age <70) should be treated with _______
autologous bone marrow
transplantation in an attempt to cure the disease
MM Tx
Older patients should receive a combination of _____
melphalan and prednisone
MM Tx
Patients who are candidates for transplants should receive ______
thalidomide (or lenalidomide) and dexamethasone
MM Tx
Hypercalcemia is treated initially with hydration and_______
loop diuretics and then with bisphosphonates such as pamidronate
____ is a proteasome inhibitor useful for relapsed myeloma or in combination with the other medications. It can be combined with steroids, melphalan, or lenalidomide (thalidomide).
Bortezomib
The overproduction of a particular immunoglobulin by plasma cells without the systemic manifestations of myeloma such as bone lesions, renal failure, anemia, and hypercalcemia.
Monoclonal Gammopathy of Uncertain Significance (MGUS)
MGUS is a very common abnormality present in ___of all patients age >50 and in ___ of those age >70
1%
3%
Dx of MGUS
Diagnosis. An elevated monoclonal immunoglobulin spike of serum protein electrophoresis
(SPEP) in amounts lower than found in myeloma
bone marrow has_____ plasma cells in MGUS
<5%
A neoplastic transformation of lymphocytes particularly in the lymph node. It is
characterized by the presence of Reed-Sternberg cells on histology which spreads in an orderly, centripetal fashion to contiguous areas of lymph nodes
Hodgkin Disease
Hodgkin disease has bimodal age distribution—one peak in the ___ and ___
20s and 60s.
HL
______ are the most common initial signs of disease
Cervical, supraclavicular, and axillary
lymphadenopathy
T or F
The staging is the same for both Hodgkin as well as non-Hodgkin lymphoma.
T
In HL
An elevated _____ level indicates an adverse prognosis
LDH
Localized disease such as stage
IA and IIA is managed predominantly with ______
radiation
All patients with evidence of “B” symptoms as well as stage III or stage IV disease are managed with chemotherapy.
The most effective combination
chemotherapeutic regimen for Hodgkin disease is _____
ABVD (adriamycin [doxorubicin], bleomycin,
vinblastine, and dacarbazine).
Hodgkin disease has several histologic subtypes. _____ has the best prognosis,
and_____has the worst prognosis
Lymphocyte-predominant
lymphocyte-depleted
The neoplastic transformation of both the B and T cell lineages of lymphatic cells.
NHL causes the accumulation of neoplastic cells in both the lymph nodes as well as more often diffusely in extralymphatic organs and the bloodstream.
The Reed-Sternberg cell is absent.
NHL
Infections such as____, ______, _______, ______predispose
to the development of NHL
HIV, hepatitis C, Epstein-Barr, HTLV-I, and Helicobacter pylori
HIV and Epstein-Barr are both more often associated with_____
Burkitt lymphoma.
HIV can also be associated with ____
immunoblastic lymphoma
HL vs NHL
The difference is that Hodgkin disease is localized to ______nodes 80–90% of
the time, whereas NHL is localized only 10–20% of the time
cervical and supraclavicular
NHL is far more likely to involve
______ as well as to have blood involvement similar to chronic lymphocytic leukemia.
extralymphatic sites
Dx of NHL
As with Hodgkin disease _______ often accompany the disease
anemia, leukopenia, eosinophilia, high LDH, and high ESR
The initial chemotherapeutic
regimen for NHL is still_____
CHOP (cyclophosphamide, hydroxy-adriamycin, oncovin [vincristine], prednisone).
CNS lymphoma is often treated with radiation, possibly in addition to ____ Relapses of NHL can be controlled with _____
CHOP.
autologous bone marrow transplantation.
NHL express CD20 antigen in greater amounts. When this occurs, monoclonal antibody ____should be used
rituximab
Prior to using R-CHOP, always test completely for ______ as rituximab can
cause fulminant liver injury in those with active disease
hepatitis B and C,
____ is an oncologic emergency caused by massive tumor cell lysis,
with the release of large amounts of potassium, phosphate, and uric acid into the systemic circulation
Tumor lysis syndrome (TLS)
Patients about to receive chemotherapy for a cancer with a high cell turnover rate–especially lymphomas and leukemias–should receive ____
prophylactic oral or IV allopurinol plus adequate IV hydration to maintain high urine output (>2.5 L/day).
___ maybe used as an alternative
to allopurinol and is reserved for those at high-risk for developing TLS
Rasburicase
The idiopathic production of an antibody to the platelet, leading to removal of platelets
from the peripheral circulation by phagocytosis by macrophages.
The platelets are bound by the macrophage and brought to the spleen, leading to low platelet counts
ITP
ITP association
ITP is often associated with lymphoma, CLL, HIV, and connective tissue disease
Dx of ITP
Thrombocytopenia is the major finding. A normal spleen on exam and on imaging
studies such as an U/S is characteristic
Platelet disorders can broadly be classified into 2 groups:
• Quantitative ______
• Qualitative _______
(low plateletccount, eg, ITP)
(normal platelet count but abnormal
platelet function, eg, Von Willebrand, Bernard Soulier)
ITP
______ is the initial therapy in almost all patients
Prednisone
____ is used in patients in whom very low platelet counts <10,000–20,000/mm3 continue to recur despite repeated courses of steroids.
Splenectomy
_____ may be used in patients with profoundlyclow platelet counts (<10,000 μL) or in patients at risk for life-threatening bleeding
IVIG or RhoGAMTM
Note that ____ may only be used in Rh-positive patients.
RhoGAM
VWD
An increased predisposition to platelet-type bleeding from decreased amounts of
____
von Willebrand factor
VWD inheritance
AD
This is the most common congenital disorder of hemostasis
VWD
Pathogenesis of VWD
vWD results in a decreased ability of platelets to adhere to the endothelial lining of blood vessels
SSx of VWD
This is mucosal and skin bleeding such as epistaxis, petechiae, bruising, and menstrual abnormalities
VWD
There is often a marked increase in bleeding after the use of _____
aspirin.
VWD
The _____which examines the ability of platelets to bind to an artificial endothelial surface (ristocetin), is abnormal
ristocetin platelet aggregation test,
Tx of VWD
_____ is used for mild bleeding or when the patient must undergo minor surgical procedures. It releases subendothelial stores of von Willebrand factor.
Desmopressin acetate (DDAVP)
The deficiency of factor ____ in hemophilia A and factor____in hemophilia B
resulting in an increased risk of bleeding
VIII
IX
Both hemophilia A and B are _____resulting in disease in
males. Females are carriers of the disease
X-linked recessive disorders
Factor-type bleeding is generally
deeper than that produced with platelet disorders. Examples of the type of bleeding found with factor deficiencies are _____
hemarthrosis, hematoma, GI bleeding, or urinary bleeding
Dx of Hemophilia
A prolonged PTT with a normal PT is expected. A factor deficiency is strongly suspected when a 50:50 mixture of the patient’s blood is created with a normal control and the PTT drops to normal. This is known as a “mixing study.”
Mild hemophilia can be treated with ___
desmopressin (DDAVP
The deficiency of vitamin K resulting in decreased production of factors ____
II, VII, IX, and X.
Vitamin K deficiency can be produced by _____
dietary deficiency, malabsorption, and the
use of antibiotics that kill the bacteria in the colon that produce vitamin K.
The antibiotics most commonly associated with Vitamin K deficiency are broad-spectrum drugs such as____
fluroquinolones, cephalosporins,
and other penicillin derivatives.
Vitamin K deficiency
Both the PT and PTT are elevated. The ___usually elevates first and more severely
PT
Tx of Vitamin K deficiency
Severe bleeding is treated with infusions of fresh frozen plasma. Vitamin K is
given at the same time to correct the underlying production defect
