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134 Cards in this Set

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___% of African-Americans carry the HbS trait, and ___% have the disease.
8%; 0.2% (1:500)
________ = defect of platelet aggregation
Glanzmann's thrombasthenia
________ disease = defect of platelet adhesion
Bernard-Soulier Disease
_____= activation of coagulation cascade leading to microthrombi and global consumption of platelets, fibrin, and coagulation factors.
DIC (Disseminated intravascular coagulation)
2 therapies for sickle cell anemia:
1.Hydroxyurea (increased HbF) 2.BM transplantation
50% of Hodgkin's cases are associated with which virus?
EBV
Alpha-thalassemia is prevalent in which populations?
Asia and Africa (A=alpha=asia=africa)
Antiplatelet antibodies and increased megakaryocytes are seen in ____.
ITP
bcl-2 activation is associated with which translocation and which lymphomas?
t(14;18) - Follicular lymphomas
bcr-abl hybrid is associated with which translocation and which leukemia?
t(9;22) - CML
Beta-Thalassemia is prevalent in which population?
Mediterranean (hint: thalassa=sea. Think, thalaSEAmia)
Blood smear of a Multiple Myeloma patient would show what?
RBCs stacked like poker chips (Rouleau formation)
Burkitt's lymphoma:'______' appearance associated with what virus? Endemic where?
'Starry sky'(sheets of lymphocytes w/ interspersed macrophages); EBV; Africa
c-myc activation is associated with which translocation and which lymphoma?
t(8;14) - Burkitt's
Causes of aplastic anemia:
Radiation, benzene, chloramphenicol, alkylating agents, antimetabolites, viral agents (Parvovirus B19, EBV, HIV), Faconi's anemia, idiopathic (immune-mediated, primary stem cell defect), acute hepatitis
Causes of platelet abnormalities:
ITP (antiplatelet antibodies/inc megakaryocytes), TTP (schistocytes), drugs, DIC(inc fibrin split products)
CFU-GM (colony forming unit-granulocyte-monocyte) gives rise to which cells?
Monocytes, neutrophils and basophils
CLL is very similar to what lymphoma?
SLL (small lymphocytic lymphoma)
Coagulation factor defects (macrohemorrage) cause: (3)
1.Hemarthroses (bleeding into joints) 2.easy bruising 3.prolonged PT and/or PTT
Common cause of macrocytic megaloblastic anemia:
Vit. B12/folate deficiency
Compare Multiple Myeloma with Waldenstrom's macroglobinemia:
Waldenstrom's also has an M spike, but large amounts of IgM are produced (not IgA or IgG), no lytic lesions
Complications often seen in homozygotes (sickle cell disease) include:
Aplastic crisis (due to B19 parvovirus infection), autosplenectomy, inc. risk of encapsulated organism infection, Salmonella osteomyelitis, painful crisis (vaso-occlusive), and splenic sequestration crisis (see color image 21)
Describe the RBCs of a parient with Hereditary spherocytosis.
RBCs are small, round, with no central pallor, less membrane therefore inc MCHC, inc RDW, inc reticulocytes
General considerations of leukemias:
Inc. number of circulating leukocytes in blood
Genetics associated with Burkitt's lymphoma:
t(8;14) c-myc gene moves next to heavy chain Ig gene (14)
Hb Barts =
Gamma-4 tetramers, lacks all 4 alpha globin genes
Hb Barts results in what?
Hydrops fetalis and intrauterine fetal death
HbH =
Beta-4 tetramers, lacks 3 alpha globin genes
Hereditary spherocytosis causes intrinsic, extravascular hemolysis due to a _____ defect.
Spectrin or ankyrin
Hereditary spherocytosis is associated with what other problems?
gallstones, SPLENOMEGALY, anemia, and jaundice
Hereditary spherocytosis is distinguished from warm antibody hemolysis by what test?
Direct Coomb's test (Hereditary spherocytosis is Coomb's negative)
Hodgkin's or Non-Hodgkin's: which is associated w/ mediastinal lymphadenopathy?
Hodgkin's
Hodgkin's or Non-Hodgkin's: which is associated with HIV & immunosuppression?
Non-Hodgkin's lymphoma
Hodgkin's or Non-Hodgkin's: which one displays a bimodal distribution?
Hodgkin's (young and old)
How does Multiple Myeloma affect calcium levels?
Destructive bone lesions cause hypercalcemia (punched-out lytic bone lesions can be seen on x-ray)
How is the diagnosis of pancytopenia made?
Bone marrow biopsy
How many alpha/beta genes are there in hemoglobin?
Alpha=4; Beta=2
In _____, the alpha globin chain is underproduced.
alpha thalassemia (1-4)
In _____, the beta chain is underproduced.
Beta-minor thalassemia (heterozygote)
In ______, the beta chain is absent.
Beta-major thalassemia (homozygote)
In anemia of chronic disease, are these values increased or decreased? 1.TIBC 2.ferritin 3.serum iron 4.storage iron in marrow macrophages 5.% sat.
1.dec. 2. inc. 3.dec. 4.inc. 5.normal
In Beta Thalassemia, cardiac failure is often due to what?
Secondary hemochromatosis (due to transfusions)
In which hemorrhagic disorder is PT increased?
DIC
In which hemorrhagic disorders is PTT increased? (4)
1.Hemophilia A 2.Hemophilia B 3.von Willibrand's disease 4.DIC
Increased or decreased in iron deficiency anemia? 1.TIBC 2.ferritin 3.serum Fe
1.TIBC=inc 2.serum Fe=dec 3.Ferritin=dec
Is Hodgkin's more common in men or women?
men (except nodular sclerosing type)
Lab findings of DIC:
increased PT/PTT, inc. fibrin split products (D-dimers), dec. platelet count
Lymphoblastic lymphoma commonly presents with what?
ALL and mediastinal mass (LL with ALL)
Lymphomas derived from the B cell lineage:
ALL (B cell), Lymphoblastic lymphoma, CLL (B), Lymphocytic lymphoma, Follicular center cell lymphoma, Immunoblastic lymphoma (B), Plasmacytoid lymphocytic lymphoma and myeloma
Lymphomas derived from the T cell lineage:
ALL (T cell) Lymphoblastic lymphoma, CLL (T) Lymphocytic lymphoma, Immunoblastic lymphoma (T), Sezary syndrome and mycosis fungoides
Macrocytic anemia is defined as MCV>____.
100
Marrow failure due to leukemia can lead to (3)
1.anemia (dec. RBCs) 2.infections (dec. WBCs) 3.hemorrhage (decreased platelets)
Microcytic, hypochromic anemia = MCV<____.
80
Name 3 coagulopathies (bleeding disorders):
1.Hemophilia A 2.Hemophilia B 3.von Willibrand's disease
Name 3 etiologies of microcytic, hypochromic anemia:
1.iron deficiency 2.Thalassemias 3.lead poisoning
Name 5 types of Non-Hodgkin's:
1.Small lymphocytic lymphoma 2.Follicular lymphoma (small cleaved cell) 3.Diffuse large cell 4.Lymphoblastic lymphoma 5.Burkitt's lymphoma
Name 6 hemorrhagic disorders.
1.Qualitative platelet defects 2.Thrombocytopenia 3. Hemophilia A (Factor VIII def.) 4. Hemophilia B (Factor IX def.) 5.Von Willibrand's disease 6.DIC
Name 9 chronic myeloid leukemias:
CML, Polycythemia Vera, Myelofibrosis, idiopathic thrombocythemia, (chronic monocytic, chronic myelomonocytic, eosinophilic, chronic erythroid) *last 4 are rare*
Nodal involvement and spread of Hodgkin's vs. Non-Hodgkin's:
Hodgkin's: localized, single group of nodes, extranodal rare, contiguous spread; Non-Hodgkin's: multiple peripheral nodes, extranodal involvement common, noncontiguous spread
Normal values: 1.TIBC 2.Serum iron 3.% sat.
1.TIBC 250-400 micro grams/dl 2.50-150 3.20-50 %
occult blood lose anemia
"dec. serum iron, inc. RBC protoporphyrin/transferrin/TIBC.
nml ferritin"
Other causes of macrocytic anemia (2)
1.Drugs that block DNA synthesis (e.g.sulfa drugs, AZT) 2.Marked reticulocytosis
pathogenesis of b+ thalassemia
promoter region mutation; splicing mutation (most common)
pathogenesis of b0-thalassemia
chain terminator mutations
Pathologic features of aplastic anemia:
Pancytopenia with normal cell morphology, hypocellular bone marrow with fatty infiltration
Peak incidence of NHL between what ages?
20-40
Philadelphia chromosome is what?
t(9;22), bcr-abl
Plasma cells in Multiple Myeloma produce large amounts of what?
IgG (55%) or IgA (25%)
Platelet abnormalities (microhemorrage) lead to:(4)
1.Mucous membrane bleeding 2.Petechiae 3.Purpura 4.Prolonged bleeding time
PMNs are hypersegmented in ____ .
Vit. B 12 and folate deficiencies
PT and PTT: which one measures the intrinsic and which one measures the extrinsic path?
PT (extrinsic); PTT (intrinsic)
Rank the prognoses of the different types of Hodgkin's:
NS and LP = excellent; MC = intermediate; LD = poor
Schistocytes are characteristic of ___.
TTP and DIC
Sickle cell heterozygotes (sickle cell trait) are relatively ____-resistant.
Malaria; (balanced polymorphism)
Symptoms of aplastic anemia:
Fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection
t(11;14) is associated with which disorder?
Mantle cell lymphoma
t(11;22) is associated with which disorder?
Ewing's sarcoma
t(15;17) is associated with which disorder?
M3 type of AML
The lymphoid stem cell gives rise to: (2)
T cells and B cells
The majority of Non-Hodgkin's involve T cells or B cells?
B cells (except lymphoblastic T cell origin)
Treatment of aplastic anemia:
Withdrawal of offending agent, allogenic bone marrow transplantation, RBC/platelet transfusion, G-CSF or GM-CSF
What are 2 indications of hemolysis?
1.decreased serum haptoglobin 2.increased serum LDH
What are constitutional signs/symptoms?
(mostly seen in Hodgkin's)low grade fever, night sweats, weight loss
What are some causes of Normocytic, normochromatic Anemia?
Acute hemorrhage, Enzyme defects (G6PD def., PK def.), RBC membrane defects (spherocytosis), Bone marrow disorders (aplasic anemia), Sickle cell, Autoimmune hemolytic anemia, Anemia of Chronic Disease
What are some other causes of DIC?
gram(-) sepsis, transfusion, trauma, malignancy, acute pancreatitis, and nephrotic syndrome
What can cause marrow failure in leukemia?
Infiltration of leukemic cells into Bone marrow (BM)
What creates a good prognosis for Hodgkin's?
Increased lymphocytes and decreased Reed-Sternberg (RS) cells
What do you find on blood smear w/ DIC?
Helmet-shaped cells and shistocytes
What does the skull look like on X-ray for sickle cell anemia?
'Crew cut' due to marrow expansion (also seen in thalassemias)
What genetics are involved with Follicular lymphoma (small cleaved cell)?
t(14;18)bcl-2 expression
What is another Beta chain mutation in which patients have a milder disease than HbSS patients?
HbC defect; patients can be HbC or HbSC(1 of each mutant gene)
What is aplastic anemia?
Pancytopenia characterized by severe anemia, neutropenia, and thrombocytopenia caused by destruction of multipotent myeloid stem cells, with inadequate production or release of differentiated cell lines.
What is compensatorily increased in both forms of Beta Thalassemia?
fetal hemoglobin; however, it is inadequate
What is found in the urine of patients with Multiple Myeloma?
Ig light chains (Bence Jones protein)
What is it called when CML transitions to AML?
Blast crisis
What is the characteristic cell of Hodgkin's lymphoma?
Reed-Sternberg cell (B-cell origin); decreased numbers of RS cells indicates a better prognosis, see image 25
What is the M spike?
Monoclonal immunoglobin spike on serum electrophoresis
What is the MOST common bleeding disorder?
von Willibrand's disease (deficiency of von Willibrand's antigen)
What is the MOST common cause of DIC?
obstetric complications
What is the MOST common primary tumor arising within bone in adults?
Multiple Myeloma
What is the shape of RBCs in sickle cell anemia?
Crescent shape
What Leukemia type has AUER rods, myeloblasts, and occurs in adults?
AML (myeloblasts)
What Leukemia type is common in children, has lymphoblasts, is MOST responsive to therapy, and may spread to CNS/testes?
ALL (pre-B or pre-T cells)
What Leukemia type is MOST commonly associated with Philadelphia chromosome (9:22 translocation->bcr-abl), myeloid stem cell proliferation, and may accelerate to AML?
CML (Myeloid stem cells)
What Leukemia type occurs in older adults, has lymphadenopathy, hepatosplenomegaly, few symptoms, indolent course, inc. smudge cells in peripheral blood smear, warm Ab autoimmune hemolytic anemia?
CLL (lymphocytes Non-antibody-producing B cells)
What mutation causes sickle cell anemia?
HbS mutation is a single aa replacement in the Beta chain (substitute normal glutamic acid with valine)
What other problems result from Multiple Myeloma?
Renal insufficiency, increased susceptibility to infections, anemia, and amyloidosis
What precipitates sickling of cells?
Low oxygen or dehydration
What test is used to confirm Hereditary spherocytosis?
Osmotic fragility test
What test is used to distinguish between immune vs. non-immune RBC hemolysis?
Direct Coomb's test
What type of cell is cancerous in Multiple Myeloma and what does it resemble?
Monoclonal plasma cell, 'fried egg' appearance
Where can leukemic cell infiltrate?
Liver, spleen, and lymph nodes
Which 2 of 6 hemorrhagic disorders have decreased platelet counts?
1.thrombocytopenia 2.DIC
Which 2 types of Non-Hodgkin's occur in children?
Lymphoblastic lymphoma, and Burkitt's lymphoma, 20% of diffuse large cell type too
Which Beta Thalassemia results in severe anemia?
Beta Thal. major (requires blood transfusions)
Which coagulation factors are a part of the intrinsic and which are a part of the extrinsic path?
PT(extrinsic):Factors II,V,VII,X; PTT(intrinsic):all factors except VII/XIII REMEMBER THE FACTORS!
Which coagulation factors are deficient in each of the Hemophilias?
Hemophilia A (factor VIII deficiency); Hemophilia B (factor IX deficiency)
Which one is associated with neurological problems, folate deficiency or Vit. B12 deficiency?
Vit. B12
Which type of Hodgkin's accounts for 6% of cases and which type is the most rare/prognosis.
Lymphocyte Predominant (LP) - 6%; Excellent prognosis
Which type of Hodgkin's commonly affects males under 35?
Lymphocyte Predominant (LP)
Which type of Hodgkin's commonly affects older males with disseminated disease?
Lymphocyte Depleted (LD)
Which type of Hodgkin's has the most Reed Sternberg cells?
Mixed Cellularity
Which type of Hodgkin's is characterized by collagen banding/lacunar cells?
NS (nodular sclerosing)
Which type of Hodgkin's is rare?
Lymphocyte Depleted (LD) - rare; Poor prognosis
Which type of Hodgkin's is the 2nd most common/prognosis? (25%)
Mixed cellularity (MC); Intermediate prognosis
Which type of Hodgkin's is the MOST common/prognosis? (65-75%)
NS (nodular sclerosing); Excellent prognosis
Which type of Hodgkin's primarily affects young adults, women>men?
Nodular Sclerosing (NS)
Which type of Non-Hodgkin's clinically presents like CLL?
Small lymphocytic lymphoma (SLL with CLL)
Which type of Non-Hodgkin's is difficult to cure?
Follicular lymphoma
Which type of Non-Hodgkin's is MOST common in children?
Lymphoblastic lymphoma (very aggressive)
Which type of Non-Hodgkin's is the MOST common type in adults?
Follicular lymphoma (small cleaved cell)
Which types of Non-Hodgkin's involve T cells?
Lymphoblastic lymphoma (immature T cells); 20% of Diffuse large cell Non-Hodgkin's (mature T cells)
With iron overload (hemosiderosis) are values increased or decreased? 1.TIBC 2.serum iron 3.%sat.
1.TIBC normal 2.Serum iron increased 3.% sat. increased (100%)