Heme, First Aid

Front 1

spherocyte

Back 1

hereditary spherocytosis, autoimmune hemolysis

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elliptocyte

Back 2

hereditary elliptocytosis

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Macro-ovalocyte

Back 3

megaolblastic anemia (also hypersegmented PMNs), marrow failure

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Helmet cell, schistocyte

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DIC, traumatic hemolysis

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Sickle cell

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sickle cell anemia

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Bite cell

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G6PD deficiency

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Teardrop cell

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myeloid metaplasia with myelofibrosis

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Acanthocyte

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spiny appearance in abetalipoproteinemia

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Target cell!!!

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HbC disease, Asplenia, Liver disease, Thalassemia

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Poikilocytes

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nonuniform shapes in TTP/HUS, microvascular damage, DIC

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Burr cell

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TTP/HUS

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Basophilic stippling

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thalassemias, anemia of chronic disease, iron deficiency, lead poisoning

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decreased serum iron, increased TIBC and decreased ferritin

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iron deficiency

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decreased TIBC, increased ferritin, increased iron in marrow macrophages

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anemia of chronic disease

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t(9;22)

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CML!! (bcr-abl hybrid)

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t(8;14)

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Burkitt's lymphoma

c-myc activation

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t(14;18)

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Follicular lymphoma

(bcl-2 activation)

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t(15;17)

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M3 type of AML

(responsive to all-trans retinoic acid!)

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t(11;22)

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Ewing's sarcoma

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t(11;14)

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mantle cell lymphoma

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Coombs negative

Osmotic fragility to confirm

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Hereditary spherocytosis

(intrinsice, extravascular hemolysis due to spectrin or ankyrin defect)

Increased MCHC and increased RDW (poss decrease MCV)

Howell Jolly bodies present after splenectomy

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decrease serum haptoglobin and increase in serum LDH

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RBC hemolysis

(direct Coombs' test is used to distinguish between immune vs. non-immune mediated RBC hemolysis)

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Microcytic anemias

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Iron deficiency

Thalassemias (target cells)

Lead poisoning

Sideroblastic anemia

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Macrocytic anemias

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megaloblastic, drugs that block DNA synthesis (sulfa, phenytoin, AZT)

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Normocytic, normochromic anemia

Back 25

acute hemorrhage, enzyme defects (G6PD deficiency...X linked and PK deficiency...AR), RBC membrane defects (hereditary sphero), bone marrow disorders, hemoglobinopathies (sickle cell disease), autoimmune hemolytic anemia; anemia of chronic disease (decreased TIBC, increased ferritin, increased storage iron in marrow macrophages)

Front 26

decreased serum iron, increased transferrin, decrease ferritin, way decreased % transferrin saturation

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iron deficiency

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decreased serum iron, decreased transferrin, increased ferritin

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anemia of chronic disease

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increased transferrin (primary) and decreased % transferrin saturation, serum iron is the same

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pregnancy, OCP use

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Increased serum iron, decreased transferrin, increased ferritin, and way increased % transferrin saturation

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hemochromatosis

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Sickle cell anemia

Back 30

HbS mutation in a single amino acid replacement in beta chain (normal glutamic acid with valine)

low O2 or dehydration precipitates sickling

complications in homozygotes (due to parvovirus B19 infection), autosplenectomy, increased risk of encapsulated organisms, Salmonella osteomyelitis, painful crisis (vaso-occlusive), renal papillary necrosis, and splenic sequestration crisis

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Hydroxyurea

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therapy for sickle cell anemia

increases HbF

(realize that newborns are initially asymptomatic because they have more HgF and less HgS)

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Crew cut on X-ray

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skull x-ray; due to marrow expansion from increase of erythropoiesis (also in thalassemias)

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HbC

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different beta-chain mutation; patients with HbC or HbSC (1 of each mutant gene) have milder disease than do HbSS patients

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alpha thalassemia

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the alpha globin chain is underproduced (as a function of number of bad genes, 1-4)

no compensatory increase of any other chains

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HbH

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beta-4 tetramers; lacks 3 alpha globin genes

H for heavier

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Hb Barts

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gamma-4 tetramers, lacks all 4 alpha globin genes

results in hydrops fetalis and intrauterine fetal death

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Alapha thalassemia is prevalent in...

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Asia and Africa

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Beta thalassemia is prevalent in...

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Mediterranean populations

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Beta thalassemia (major vs. minor)

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Minor (hetero): beta chain is underproduced

Major (homo): beta chain is absent

both cases: fetal hemoglobin production if compensatorily increased but is inadequate

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Beta thalassemia results in...

Back 40

severe anemia requiring blood transfusions

cardiac failure due to secondary hemochromatosis

marrow expansion ('crew cut' on skull x-ray) leads to skeletal deformities

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Sepsis (gram negative), Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion

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causes of DIC

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Bernard Soulier Disease

Back 42

defect in platelet adhesion

decrease in GP Ib

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Glanzmann's thrombasthenia

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defect of platelet aggregation

decrease in GP IIb-IIIa

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PT (extrinsic)

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factors II, V, VII, and X

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PTT (intrinsic)

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all factors except VII

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What test is increased for Hemophilia A or B?

Back 46

PTT!!!

(nothing else)

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Distinctive tumor giant cell seen in Hodgkin's disease

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Reed Sternberg cells

(necessary but not sufficient for a diagnosis of Hodgkin's disease)

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binucleate or bilobed cell with the 2 halves as mirror images ('owl eyes')

prominent nucleoli surrounded by lymphocytes and other reacting inflammatory cells

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Reed Sternberg cell

in Hodgkin's disease

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CD 30+ and CD 15 + B cell origin

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RS cells

(presence of Reed Sternberg cells in Hodgkin's lymphoma)

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Localized, single group of nodes; extranodal is rare; contiguous spread

Back 50

Hodgkin's lymphoma

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Constitutional ('B') signs/symptoms - low grade fever, night sweats, weight loss

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Hodgkin's lymphoma

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A good prognosis of Hodgkin's in...

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increased lymphocytes and decreased RS cells

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Where do you find lymphadenopathy in Hodgkin's?

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Mediastinal lymphadenopathy

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50% cases are associted with EBV; bimodal distribution - young and old; more common in men except for nodular sclerosing type

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Hodgkin's lymphoma

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Associated with HIV and immunosuppression

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Non-hodgkin's lymphoma

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Multiple, peripheral nodes; extranodal involvement common; noncontinguous spread

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Non-Hodgkin's

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No hypergammaglobulinemia, fewer constituitional signs/symptoms and peak incidence 20-40 years of age

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Non-Hodgkin's

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Nodular sclerosing

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most common type of Hodgkin's lymphoma

65-75%

more lymphocytes than RS cells, thus has an excellent prognosis

collagen banding; lacunar cells; women > men; primarily young adults

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Mixed cellularity Hodgkin's lymphoma

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25%

intermediate prognosis

numerous RS cells (lots of lymphs too)

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Lymphocyte predominat Hodgkin's

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6%

excellent prognosis (because more lymphocytes)

< 35 year old males

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Lymphocyte depleted Hodgkin's

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rare!!

pooooor prognosis

older males with disseminated disease

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Small lymphocytic lymphoma

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Non-Hodgkin's

Adults

B cells

Like CLL, with focal masses; low grade and indolent

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Follicular lymphoma (small cleaved cell)

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Non-Hodgkin's lymphoma

Adults

B cells

t(14;18): bcl-2 expression

Most common!! (adult)

difficult to cure; indolent course; bcl-2 inhibits apoptosis

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t(14;18)

Back 64

bcl-2 expression!!

Follicular lymphoma (small cleaved cell)

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Diffuse large cell lymphoma

Back 65

Non-Hodgkin's lymphoma
Usually in older adults, but 20% occur in children

80% B cells
20% T cells

aggressive, but up to 50% are curable

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Mantle Cell Lymphoma

Back 66

Adults

B cell

t(11;14)

Poor prognosis!!

CD5+

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t(11;14)

Back 67

Mantle cell lymphoma

aggressive, older men

activates cyclin D (bcl-1)

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Lymphoblastic lymphoma

Back 68

most often children!!!

presents with ALL and mediastinal mass

T cells (immature)

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Most common in children non-Hodgkin's lymphoma

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lymphoblastic lymphoma

T cells (immature)

commonly presents with ALL and mediastinal mass

very aggressive T cell lymphoma

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Burkitt's lymphoma

Back 70

most often in children

B cell

t(8;14) c-myc gene moves to heavy chain Ig gene (14)

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'Starry sky' appearance (sheets of lymphocytes with interspersed macrophages)

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Burkitt's!!

t(8;14) c-myc

Front 72

associated with EBV

t(8;14)

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Burkitt's!!

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jaw lesion in endemic form in Africa

pelvis or abdomen in sporadic form

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Burkitt's

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most common malignancy in children

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ALL!!

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General stuff about leukemias

Back 75

increase number of circulating leukocytes in blood

bone marrow infiltrates of leukemic cells

marrow failure can cause anemia (decrease RBC), infections (decrease WBC) and hemorrhage (decrease platelets)

leukemic cell infiltrates in liver, spleen and lymph nodes are common

bone marrow infiltrated with leukemic cells, often with encroachment on normal hematologic cell development

full bone marrow!

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age for ALL

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less than 15

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age for AML

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5-40

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age for CML

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30-60

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age for CLL

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greater than 60

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Leukemoid reaction

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increase with left shift (80% bands) and increase in leukocyte alkaline phosphatase

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TdT +

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marker of pre-T and pre-B cells in ALL

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ALL

Back 82

children; lymphoblasts (pre-B or pre-T)...TdT+

most responsive to therapy

may spread to CNS and testes

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AML

Back 83

auer rods (abnormal fusion of primary azurophilic granules)

myeloblasts

adults

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CLL

Back 84

older adults (men > 60)

lymphocytes, non-antibody producing B cells

lymphadenopathy, hepatosplenomegaly; few symptoms; indolent course

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increase smudge cells in peripheral blood smear

Back 85

CLL

Front 86

warm antibody autoimmune hemolytic anemia and similar to SLL (small lymphocytic lymphoma)

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CLL!!

survival is about 3-7 years

also, hypogammaglobulin causes increase susceptibility to infection

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Myeloid stem cell proliferation

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CML

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presents with increased neutrophils and metamyelocytes; splenomegaly; may accelerate to AML (blast crisis)

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CML

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very low leukocyte alkaline phosphatase (vs. leukemoid reaction)

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CML!

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General about acute leukemias

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blasts predominate; children or elderly; short and drastic course

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General about chronic leukemias

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more mature cells; midlife age range; longer, less devastating course

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Auer bodies

Back 92

peroxidase-positive cytoplasmic inclusions in granulocytes and myeloblasts

primarily seen in acute promyelocytic leukemia (M3)

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what can treatment of AML M3 do?

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release Auer Rods!! and progress to DIC

(treatment of AML M3 is with all trans retinoic acid...t(15;17))

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Hairy cell leukemia

Back 94

mature B cell tumor in the eldery

cells have filamentous, hair-like projections

stains TRAP (tartrate-resistant acid phosphatase) positive

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Histiocytosis X

Back 95

caused by Langerhans cells from the monocytic lineage that infiltrate the lung

Birbeck granules (tennis rackets on EM)

primarily affects young adults

worse with smoking

(characterized by proliferation of histiocytic cells that closely resemble the Langerhans cells of the epidermis)