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239 Cards in this Set

  • Front
  • Back
which hormone stimulates the maturation of red blood cells?
erythropoietin
what is the lifespan of a RBC?
120 days
Name the dz: back pain, decreased Hb, increased creatinine?
mulitple myeloma
with acute blood loss, what effect is there on platelets and WBCs?
there may be a reactive thrombocytosis and leukocytosis
What Dur(T)ie Salmon stage do you see physical findings of multiple myeloma?
Stage III
What indicates poor prognosis in multiple myeloma?
increased creatinine, elevated beta-2 microglobulin
What is MGUS?
m-spike w/o neoplasm or symptoms
what shape are erythrocytes?
biconcave discs
t/f... erythrocytes do not have organelles
true
which cells are the last in the erythroid line to have a nucleus?
normoblasts
which type of malaria is associated with the greatest morbidity and mortality
P falciparum
what does the haemopoietic stem cell look like?
small to intermediate sized lymphocyte
What cells are likely to be found in the peripheral blood of a CML patient?
immature granulocytes at various stages
what is the source of erythropoietin?
kidney
what metals are required for normal haemopoiesis?
iron, manganese, cobalt
which vitamins are required for normal haemopoiesis?
B12, folic acid, C, E, B6, thiamine, riboflavin, pantothenic acid
what is the most common cause of anaemia?
iron deficiency
what is the laboratory hallmark of iron deficiency?
microcytosis
what is the definition of microcytosis?
mean corpuscular volume less than 80 femtolitres
what is the best measurement of overall iron stored in the body?
serum ferritin
With low grade lymphoma, what are common lab findings?
Nothing - usually pretty normal, just find messed up things in biopsy
In promyelocytic leukemia (a AML variant), what are some typical findings?
Lots of promyelocytes in marrow + blood; often have hypo or hyper granules, odd nuclei; maybe thromboses or hemorrhage. Treat w/ ATRA
what is the most common cause of macrocytosis?
excess alcohol consumption
which deficiency can cause profound neurological damage?
Vitamin B12
what is Vit B12 deficiency most commonly caused by?
poor absorption from the bowel (often associated with pernicious anaemia)
Hodgkin's and non-Hodgkin's lymphomas are often described in term of what?
Hodgkins: lymphocyte morphology +/or phenotype; NH: morphology +/or phenotype, + overall pattern (diffuse vs follicular)
what substance is secreted by gastric cells?
intrinsic factor
What are peripheral blood and bone marrow findings for a patient w/ B-CLL?
Small, mature, round lymphocytes; express B cell antigens, some Ig; >30% of cells in marrow
where does folic acid absorption occur?
jejunum
which deficiency affects the peripheral nervous system only?
folate
which two conditions are associated with reduced haemoglobin concentration of the erythrocytes and are characterised by microcytic hypochromic erythrocytes?
iron deficiency and thalassaemia
what is the best screening test for iron deficiency?
serum ferritin
What is MGUS?
Dx for an asymptomatic patient w/ a monoclonal protein, but you can't find the cells that are making it. 20-30% later develop PCM (no bone lysis)
Is nodular lymphocyte predominant HL a form of classical? Is nodular sclerosis HL?
Nodular lymphocyte is not: expresses CD45 + CD20, + cell = H+L (popcorn); nodular sclerosis: CD15 + 30+, no B or T , lots of collagen. Main difference: the classicals have HRS, non doesn't
which two conditions result in rapid falls in haemoglobin concentration?
haemolysis and blood loss anaemia
What is the bad cell in Burkitt lymphoma? What pattern does it present with?
small non cleaved lymphocyte or follicular B-blast. Rarely has follicular pattern
What are the most common types of NHL? What are the most aggressive?
B cell (except in kids); aggressive: precursor B or T cell lymphoblastic leukemia/lymphoma, and Burkitt's
where is haptoglobin produced?
liver
what are the common causes of chronic blood loss?
In the United States: menorrhagia, gastrointestinal bleeding.
Worldwide: hook worm disease (necator americanus)
which two pathways in the white matter of the spinal cord are affected by B12 deficiency?
the dorsal columns and the lateral corticospinal tract
List the common symptoms of anemia as well as the symptoms of severe anemia.
Common: pallor, fatigue, dyspnea on exertion.
Severe: angina pectoris, glossitis, gastritis, koilonychia, and Plummer-Vinson syndrome.
What are the iron study patterns in iron deficiency anemia?
Serum iron is low.
TIBC is elevated.
Serum ferritin is low.
what is anaemia?
reduction in red cell number, haemoglobin or haematocrit
why does haemoglobin reduce in pregnancy?
haemodilution
what is the normal range for reticulocytes?
0.5% - 1.5%
what fraction of a red blood cell is normally central pallor?
1/3
t/f... reticulocytes contains some RNA
true
what are the iron study patterns in the anemia of chronic disease?
Serum iron is low
TIBC is low.
Serum ferritin is elevated.
what is required for red cell production?
erythropoietin, vit B12, folic acid
where is erythropoietin produced?
kidney (and to a lesser extent the liver)
Which antibodies mediate warm antibody autoimmune hemolytic anemia versus cold agglutinin disease?
IgG: warm
IgM: cold
what are the haematinics?
iron, B12, folate
What preventative measure can be taken to prevent erythroblastosis fetalis?
administer anti-D IgG antiserum to D negative mothers at the time of delivery of a D positive child. this causes fetal red cells to be removed from maternal circulation.
What are the possible causes of erythroblastosis fetalis?
Maternal alloimmunization to fetal Rh antigens.

ABO incompatibility.
What is the pathophysiology of paroxysmal nocturnal hemoglobinuria?
Defect in the PIG-A gene causes impaired synthesis of the GPI anchor, which fixes CD55, CD59, and CD8 proteins to the cell surface. These proteins protect red cells from compliment mediated lysis.
What are the possible molecular defects in hereditary spherocytosis? What is its inheritance?
Spectrin, ankyrin, protein 4.1 or other RBC skeletal proteins.

Autosomal dominant.
What is the pathophysiology of G6PD deficency? What is its inheritance?
Lack of G6PD reduces the body's ability to deal with oxidative stress. G6PD is an X-linked disorder.
what effect does anaemia have on viscosity of blood?
anaemia reduces viscosity of blood
what is the effect of reduced O2 delivery to tissues on blood vessels?
dilatation
which deficiency leads to both peripheral and central neurological signs?
B12
will deficiency of the haematinics be associated with a low or high reticulocyte count?
low
will haemolysis be associated with a low or high reticulocyte count?
high
What are the six symptoms of sickle cell anemia?
severe hemolytic anemia
chronic leg ulcers
vaso-occlusive crises
auto splenectomy
aplastic crises (parvovirus)
infectious complications (salmonella osteomyelitis)
What are six clinical and laboratory signs of beta thalassemia?
severe anemia
severe splenomegaly
distortion of skull facial bones and long bones
microcytosis, hypochromia, target cells
increased Hemoglobin F
generalized hemosiderosis
what type of anaemia will occur with marrow suppression/aplasia?
normocytic, normochromic
what type of anaemia do defects in Hb synthesis cause?
microcytic hypochromic
What is the spectrum of clinical abnormalities in alpha thalassemia according to the number of deletions?
one deletion: no abnormalities
two to three deletions: mild to moderate thalassemic symptoms
four deletions: hydrops fetalis
What is hepcidin?
A peptide hormone that decreases both intestinal iron absorption and the release of iron from macrophages. In anemia of chronic disease, proinflammatory cytokines induce hepatic synthesis of hepcidin. This causes serum iron to be low despite normal or even elevated iron stores (ferritin).
Where does the antigen-independent phase of plasma cell development occur?
bone marrow (heavy and light chain rearrangement, surface Ig expression)
Where does the antigen-dependent phase of plasma cell development occur?
spleen, LN (antigen presentation and somatic hypermutation)
when does rouleaux occur?
increased plasma proteins e.g. myeloma
which leakaemia is associated with Auer rods?
AML
What is plasmacytoma?
- isolated plasma cell tumor, or part of MM
- occurs in different sites (in skeletal or soft tissue)
- skeletal involvement more likely progress to MM
- local Tx with radiation
what is the commonest form of anaemia?
iron deficiency
what are the two stores of iron in the body?
ferritin, haemosiderin
t/f... there is no effective excretion mechanism for iron
true
how is iron balance controlled?
iron absorption
what regulates iron absorption?
mucosal cells of proximal small intestine
what are the two pathways for dietary iron absorption?
iron attached to haem, iron in ferrous form
What are the two main clinical features of acute lymphoblastic leukemia (ALL)?
1. bone marrow failure
2. organ infiltration
What does bone marrow failure result in?
1. anemia: pallor, lethargy
2. neutropenia: fever, malaise, infections
3. thrombocytopenia: bruises, bleeding
what reduces ferric iron to ferrous form?
Dcytb (duodenal cytochrome b1)
what is the role of DMT1 (divalent metal transporter) in dietary iron absorption?
apical uptake
what transfers iron into the plasma from the enterocyte?
ferroportin (transfer is facilitated by hephaestin)
what is hepcidin?
peptide hormone secreted by liver
what is the function of hepcidin?
decreases functional activity of ferroportin
how does intestinal absorption relate to liver hepcidin expression?
absorption varies inversely with expression
which cells are destroyed by antibodies in pernicious anaemia?
parietal cells
what is the average daily loss of iron in men and non-menstruating women?
1 mg/day
Why would gout or renal impairement be a feature of CML?
excessive purine breakdown causing hyperuricemia
What are symptoms of myelodysplasia?
anemia
infections
easy bruising/bleeding
what is the most important lab marker of iron deficiency?
serum ferritin
what effect does iron deficiency have on serum transferrin?
raised
what percentage of oxygen in the blood is dissolved?
1.5%
You have determined that there is bone marrow failure (anemia, neutropenia, thrombocytopenia) and organ infiltration (sore bones) in a 4 year old girl. What is the most likely diagnosis?
ALL
what is the most common cause of vitamin B12 deficiency?
malabsoption due to inadequate intrinsic factor,disorders of terminal ileum; malnutrition due to chronic alcoholism
what is the Bohr effect?
oxygen carrying capacity of Hb at a particular PO2 is decreased by increased PCO2
what is the affinity of CO for Hb compared to oxygen?
200 times
what type of anaemia occurs in thyroid disease?
macrocytic
what volumes define microcytic, normocytic and macrocytic?
microcytic: <80 fl
normocytic: 80-99 fl
macrocytic: >99 fl
what type of anaemia occurs with impaired DNA synthesis?
megaloblastic (vit B12 or folate deficiency)
list some blood film changes in megaloblastic anaemia
oval macrocytes, aniso/poikilocytosis, hypersegmented neutrophils, may have low WCC and low platelets
how long do body stores of vitamin B12 last?
3-4 years
what is vitamin B12 bound to in the plasma?
transcobalamin (TC II)
where is vitamin B12 absorbed?
terminal ileum
what secretes intrinsic factor?
gastric parietal cells
what does B12 bind to in the stomach to travel to the terminal iluem for absorption?
intrinsic factor
which columns of the spinal cord are affected in B12 deficiency?
posterior and lateral
which peripheral fibres are affected in B12 deficiency?
peripheral sensory nerves
t/f... inadequate intake is a common cause of vitamin B12 deficiency
false, it is a very rare cause (only in true vegans)
What are some clinical features of Hodgkin's lymphoma?
1. painless, asymmetrical, firm superficial lymph nodes
2. splenomegaly (50%)
3. mediastinal enlargement
4. constitutional symptoms
what is pernicious anaemia?
severe lack of intrinsic factor due to autoimmune disease affecting gastric parietal cells
is pernicious anaemia more common in men or women?
women (M:F 1:1.6)
which antibody is highly specific for pernicious anaemia?
anti-intrinsic factor (found in 95% patients)
how long do the body stores of folate last?
3-4 months
where is the body's store of folate?
liver
where is folic acid absorbed?
proximal jejunum
t/f... folic acid is absorbed via specific receptors
false, folic acid is absorbed via concentration dependent diffusion (B12 is absorbed via specific receptors)
when is there an increased need for folic acid?
pregnancy, prematurity, haemolysis, malignancy
which disease causes malabsorption of folate?
Coeliac disease
name some antifolate drugs
methotrexate, anticonvulsant, trimethoprim, alcohol
which measure of folate tests body stores not just recent intake?
red cell folate
name three situations in which serum folate will be raised
severe vitamin B12 deficiency
folic acid therapy
sample haemolysed
when might red cell folate be falsely normal?
blood transfusion
increased reticulocytes
when will the peak reticulocyte response be observed after starting therapy for megaloblastic anaemia?
6-7 days
how long after beginning treatment for megaloblastic anaemia will the bone marrow be normoblastic?
48 hours
at what rate will the Hb rise after commencing treatment for megaloblastic anaemia?
Hb should rise by 10g/L per week
which cells are destroyed by antibodies in pernicious anaemia?
parietal cells
What are constitutional symptoms?
fever
pruritis (itching)
weight loss
weakness
fatigue
anorexia
What are some clinical features of non-Hodgkin's lymphoma?
1. superficial (asymmetrical) lymphadenopathy
2. constitutional symptoms
3. oropharyngeal involvement
4. anemia
5. hepato/splenomegaly
6. other organ involvement
A patient presents with asymmetric matted lymph nodes. Is it more likely to be leukemia or lymphoma?
Lymphoma
A patient presents with asymmetric lymph nodes, constitutional symptoms, splenomegaly and a sore throat. Is it more likely Hodgkin's or non-Hodkin's?
non-Hodgkin's sometimes presents with oropharyngeal symptoms
t/f... reduced affinity of oxygen for Hb is equivalent to a shift to the left of the O2-Hb curve
false, it is a shift to the right
list 4 factors that will decrease oxygen affinity for Hb
increased PCO2
acidosis
increased temp
increased 2,3-DPG
A 60 year old patient presents with sudden severe anemia, thrombocytopenia and enlarged bleeding gums. What is the most likely diagnosis?
AML
In which hematologic malignancy is hypermetabolism a major feature?
CML
What are some clinical features of chronic lymphocytic leukemia (CLL)?
1. symmetric enlargement of cervical, axillary or inguinal nodes
2. anemia (thrombocytopenia)
3. splenomegaly (later stages)
4. immunosuppression
how much iron do pregnany women lose every day?
3.5 mg/day
what is the average daily iron loss in menstruating women?
2 mg/day
How can abnormal platelet function present?
bruising, epistaxis, menorrhagia, hemorrhage
what is the sole physiologic means of iron transport?
transferrin
How does hypermetabolism present?
weight loss, lassitude, anorexia, night sweats
What are clinical features of chronic myeloid leukemia (CML)?
1. hypermetabolism
2. splenomegaly
3. anemia
4. abnormal platelet function
5. gout/renal impairement
What is characteristic of the M4/M5 variants of AML?
gum hypertrophy
skin involvement
CNS disease
What is characteristic of M3 variant of AML?
disseminated intravascular coagulation (DIC)
What are clinical features of acute myeloid leukemia (AML)?
similar to ALL
mostly anemia, thrombocytopenia
What does organ filtration result in?
tender bones
lymphadenopathy
hepato/splenomegaly
meningeal syndrome (headache, nausea, blurred vision)
t/f... haem iron absorption is enhanced by ascorbate and meat and inhibited by phytates, bovine milk, tea and coffee
false, this is true for non-haem iron absorption (haem iron absorption is unaffected by composition of diet)
How do you Tx multiple myeloma?
1) alkyating agents (melaphalan, prednisone)
2) nonalkylating agents (vincristine, adriamycin, decadron)
3) thalidomide, revlimib
What are the si/sx of hypercalcemia?
- ab pain, constipation
- confusion, lethargy
- renal insufficiency (calcium is diuretic)
How does multiple myeloma cause hypercalcemia?
osteoclast activating factors (mostly IL-1)
Criteria for Dx Multiple Myeloma:
1) M-spike in serum or urine
2) >10% clonal plasma cells in BM
3) end-organ damage
4) hypercalcemia
5) lytic bone lesions
6) anemia
7) renal insufficiency
* hyperviscosity, amyloidosis
How does multiple myeloma present?
- back pain, osteoporosis
- fatigue, anemia
- high ESR
- proteinuria
What is multiple myeloma?
- diffuse plasma cell growth
- end organ damage
t/f... most people with anaemia receive a transfusion
false, the only indication for transfusion is tissue hypoxia (severe anaemia)
What is MGUS?
- low grade clonal proliferation (<10% plasma cells in BM)
- asymptomatic
- can convert to MM (1%)
All the plasma cell disorders occur during the transition from _____ cells to plasma cells.
memory
what do oval macrocytes indicate?
B12 or folate deficiency
how many lobes does a hypersegmented neutrophil have?
>5
what do target cells indicate?
hypochromia
what is the normal size of a RBC compared to a lymphocyte?
RBC should be size of lymphocyte nucleus
What are possible causes of mechanical disruption leading to hemolysis? What is seen on the peripheral smear?
aortic valve prosthetics
disseminated intravascular coagulation
thrombotic thrombocytopenic purpura

smear shows schistocytes or helmet cells
list 3 causes of macrocytic normochromic anaemia
B12 deficiency, folate deficiency, myelodysplasia, alcohol consumption
What is sickle-cell thalassemia?
Co-inheritance of hemoglobin S and thalassemia of the beta globin gene. Clinically similar to sickle cell anemia but less severe.
list some causes of haemolysis
immune attack on red cells (autoimmune, drugs e.g. methyldopa, alloimmune e.g. incompatible red cell transfusion, Rh disease), abnormal red cell membrane (HS), abnormal red cell metabolism (G6PDH deficiency), mechanical (malfunctioning prosthetic heart valve, DIC, malignancy)
Specifically, what is the genetic defect in hemoglobin S disorders?
A point mutation on chromosome 11 in codon six of the beta globin gene results in a substitution of valine for glutamic acid.
What percentage of African-Americans carry the hemoglobin S gene? What advantages does the gene confer?
Approximately 7%
The hemoglobin S gene confers resistance to malarial infection (Plasmodium falciparum).
What is the inheritance of pyruvate kinase deficiency anemia?
autosomal recessive.
How can you differentiate the presentation of pyruvate kinase deficiency from G6PD deficiency?
In contrast to G6PD deficiency anemia, pyruvate kinase deficiency anemia is chronic and sustained.
What are sources of oxidative stress that can cause an episode of hemolysis in G6PD?
drugs such as primaquine (anti malarial), sulfonamides, other oxidant drugs.
fava beans
infection
what are the signs of anaemia?
pallor (conjunctival, palmar crease), cardiac decompensation (tachycardia, postural hypotension, CCF)
list the symptoms of anaemia
tiredness, headaches, dizziness, SOB, palpitations, angina (with pre-existing heart disease)
t/f... cardiac output increases in response to anaemia
true
what happens to senescent RBCs?
removed to the spleen by macrophages
cold agglutinin disease is a complication of what diseases?
infectious mononucleosis and mycoplasma pneumonia.

it may be a chronic complication of lymphoid neoplasms.
what effect does hypoxia have on the production of EPO?
production is increased by hypoxia
What is polychromatophilia?
increased numbers of larger red cells that stain with a bluish cast. These are premature RBCs and would likely stain as reticulocytes.
what are some causes of aplastic anemia?
Toxic exposure
dysfunction of cytotoxic T cells
radiation exposure
chemicals such as benzene
therapeutic drugs such as chloramphenicol, sulfonamides, gold salts, chlorpromazine, antimalarial drugs, and alkylating agents.
Viral infection by parvovirus or hepatitis C.
Name six causes of folate deficiency anemia.
dietary deprivation in alcoholics or dieters.
Pregnancy
phenytoin or oral contraceptive therapy
folate acid antagonist chemotherapy
relative deficiency in hemolytic anemia
intestinal malfunction due to sprue or Giardia
How do you distinguish folate deficiency from vitamin B12 deficiency?
There are no neurologic abnormalities in folate deficiency.
Name eight causes of vitamin B12 deficiency megaloblastic anemia.
pernicious anemia, total gastric resection, disorders of the distal Ilium, a strict vegetarian diet, intestinal malabsorption syndromes, blind loop syndrome, broad-spectrum antibiotic therapy, Diphyllobothrium latum infestation (fish tapeworm)
What are the three peripheral blood findings in macrocytic anemias? What is seen in the bone marrow?
pancytopenia, global macrocytosis, hypersegmented neutrophils.

the bone marrow shows megaloblastic hyperplasia.
How do you distinguish iron deficiency anemia from beta thalassemia minor?
In beta thalassemia minor, the alpha-2 hemoglobin is increased.
What groups are at risk for dietary deficiency of iron?
Premature infants: human milk is low in iron, and newborn iron is depleted within six months. Pregnant women may also require iron supplements.

The elderly are also at risk of dietary deficiency.
What are the differences between sporadic and endemic Burkitt's?
Sporadic: in US, mostly in Peyer's patches, rarely have EBV; endemic = Africa, in jaw + abdomen, most have EBV
what are some causes of haemolytic anaemia?
hereditary spherocytosis, G-6-PDH deficiency, sickle cell anaemia, thalassaemia, immune disorders
what are the clinical features of haemolysis?
scleral jaundice, splenomegaly, increased reticulocytes, elevated LDH, asbence of serum haptoglobin, haemosiderin in urine
What are the malignant cells in NHL?
T or B cells that were screwed up at a certain developmental point so they recapitulate morph + immunology of some cell precursor (from lymph node stage)
What is smoldering myeloma?
A more indolent form when findings are not sufficient for outright PCM diagnosis. Pt often asymptomatic, may be stable for years
What does a PCM cell look like? How does the bone marrow look?
Cell: varying degrees of atypia; maybe multinucleated, or anaplastic. BM: may be patchy; see nodules or infiltrate of pc, trabeculae look thin + sparse
How do plasma cell myelomas lead to bone lesions?
PCM cells secrete osteoclast activating factor, which includes several substances like TNF-B + IL-1.
What are typical findings for a patient w/ plasma cell myeloma?
moderate to severe normochromic normocytic anemia; maybe neutro + thrombocytopenia; monoclonal protein in serum +/or urine; Bence Jones, inc calcium, renal abnormalities
What is the most common type of chronic lymphoid leukemia?
B - CLL
+ NSE in cells indicates which disease?
Chronic myelomonocytic leukemia; a CMPD (monocytes) + MDS (other cells lines) Must have >1000 monos
how does B12 deficiency result in neurological deficits?
fatty deposits accumulate in the myelin and predisposing to myeline breakdown and APs are slowed
What indicates AML?
Auer rods; MPO +; cells having antigen for CD13, 34, HLA-DR
hypersegmented neutrophils are a feature of which type of anaemia?
amegaloblastic anaemia
which deficiencies result in megaloblastosis?
vitamin B12 and folate
SLL is the nodal based disease comparative to ________
B-CLL (SLL = small lymphocytic lymphoma) cells =small, mature, express CD19, 20, light chain
What are some typical findings in a patient with plasma cell myeloma?
High lymphs; RBC rouleaux in smear; high protein, low albumin levels (suggest abnormal protein); hypercalcemia; SPEP = monoclonal spike; odd Ig levels; hypercellular marrow w/ lots of plasma cells
What antigens are expressed by cells in nodular sclerosing Hodgkin lymphoma?
CD15, CD30, no CD45, no CD20
What are physical findings with nodular sclerosing Hodgkin lymphoma?
effaced lymph node with lots of fibrosis and slightly nodular appearance; RSC surrounded by mileau (lymph, plasma, eos)
What is typical bone marrow appearance for a patient with PV?
Excess of RBC precurors and megakaryocytes; lack of iron stores,
All CMPD have an excess of what cell type in the bone marrow?
Megakaryocytes, which produce growth factors for fibros -> fibrosis
What are the blast %s that differentiate the phases of CML?
<5 = chronic; 5-19 = accelerated; >20 = blast
t/f... commitment to a cell lineage is reversible
false, this process is irreversible
What disease is characterized by absolute basophilia?
CML
where does haemopoiesis take place in the foetus?
in the liver and spleen up to 7 months gestation and in the bone marrow after that
How do you treat hyperviscosity?
plasmapheresis
Clinical features of waldenstrom?
hyperviscosity related, fatigue, lymphadenopathy
Pathophys of waldenstrom macroglob?
malignant lymphoma w/small lymphocytes that excrete IgM
what are the last cells of the erythroid line that can synthesise haemoglobin?
reticulocytes
what are the last cells capable of division in the erythroid line?
polychromatophilic erythroblasts
what deficiency is the result of chronic blood loss?
iron
Physical findings in multiple myeloma?
pain, fatigue (anemia and renal failure), infxn, mental status changes, hyperviscosity
how is increased production of RBCs by the marrow achieved?
expansion of the volume of red marrow and by shortening of transit time for red cell maturation
Major criteria for dx of multiple myeloma?
BM plasmacytosis >30%, M-spike, Bence-Jones, plasmacytoma
What is a Bence-Jones protein?
kappa or lambda light chains in urine found in multiple myeloma
What are the two main clinical features of acute lymphoblastic leukemia (ALL)?
1. bone marrow failure
2. organ infiltration
What does bone marrow failure result in?
1. anemia: pallor, lethargy
2. neutropenia: fever, malaise, infections
3. thrombocytopenia: bruises, bleeding
What does organ filtration result in?
tender bones
lymphadenopathy
hepato/splenomegaly
meningeal syndrome (headache, nausea, blurred vision)
What are clinical features of acute myeloid leukemia (AML)?
similar to ALL
mostly anemia, thrombocytopenia
What is characteristic of M3 variant of AML?
disseminated intravascular coagulation (DIC)
What is characteristic of the M4/M5 variants of AML?
gum hypertrophy
skin involvement
CNS disease
What are clinical features of chronic myeloid leukemia (CML)?
1. hypermetabolism
2. splenomegaly
3. anemia
4. abnormal platelet function
5. gout/renal impairement
How does hypermetabolism present?
weight loss, lassitude, anorexia, night sweats
How can anemia present?
pallor, dyspnea, tachycardia
How can abnormal platelet function present?
bruising, epistaxis, menorrhagia, hemorrhage
Why would gout or renal impairement be a feature of CML?
excessive purine breakdown causing hyperuricemia
What are symptoms of myelodysplasia?
anemia
infections
easy bruising/bleeding
What are some clinical features of chronic lymphocytic leukemia (CLL)?
1. symmetric enlargement of cervical, axillary or inguinal nodes
2. anemia (thrombocytopenia)
3. splenomegaly (later stages)
4. immunosuppression
What are some clinical features of Hodgkin's lymphoma?
1. painless, asymmetrical, firm superficial lymph nodes
2. splenomegaly (50%)
3. mediastinal enlargement
4. constitutional symptoms
What are constitutional symptoms?
fever
pruritis (itching)
weight loss
weakness
fatigue
anorexia
What are some clinical features of non-Hodgkin's lymphoma?
1. superficial (asymmetrical) lymphadenopathy
2. constitutional symptoms
3. oropharyngeal involvement
4. anemia
5. hepato/splenomegaly
6. other organ involvement
A patient presents with asymmetric matted lymph nodes. Is it more likely to be leukemia or lymphoma?
Lymphoma
A patient presents with asymmetric lymph nodes, constitutional symptoms, splenomegaly and a sore throat. Is it more likely Hodgkin's or non-Hodkin's?
non-Hodgkin's sometimes presents with oropharyngeal symptoms
You have determined that there is bone marrow failure (anemia, neutropenia, thrombocytopenia) and organ infiltration (sore bones) in a 4 year old girl. What is the most likely diagnosis?
ALL
A 60 year old patient presents with sudden severe anemia, thrombocytopenia and enlarged bleeding gums. What is the most likely diagnosis?
AML
In which hematologic malignancy is hypermetabolism a major feature?
CML