Things you Must Know – ANEMIA
Iron-Deficiency Anemia
-Most important cause: GI bleeding
-Microcytic, hypochromic anemia
-Increased anisocytosis and poikilocytosis
-Abnormal iron studies
Megaloblastic Anemia
-Defective DNA synthesis
-Nuclear/cytoplasmic asynchrony
-Decreased B12/folate
-Macrocytic anemia with oval macrocytes and
hypersegmented neutrophils
Hereditary Spherocytosis
-Tons of spherocytes
-Spectrin defect
-Splenectomy is curative
Warm Autoimmune Hemolytic Anemia
-IgG
-Spleen
Spherocytes
Cold Autoimmune Hemolytic Anemia
-IgM, complement
-Intravascular hemolysis
-Agglutination
Hemoglobinopathies
-Qualitative abnormality in Hgb
-Sickle cell anemia most important one
-Sickle cells  hemolysis, vaso-occlusion
Thalassemia
-Quantitative defect in hemoglobin
-Can’t make enough α or β chains
-Variable disease severity
-Hypochromic, Microcytic anemia with increased RBC
and target cells
Glucose-6-Phosphate Dehydrogenase Deficiency
-↓ G6PD → ↑peroxides → cell lysis
-Oxidant exposure
-Bite cells (removal of Heinz bodies by spleen)
-Self-limiting
Microangiopathic Hemolytic Anemia
-Schistocytes
-Find out WHY!
Anemia of Blood Loss
-Cause: traumatic, acute blood loss
-At first, hemoglobin is normal!
-After 2-3 days, see reticulocytes
-Chronic blood loss is different (it causes iron
deficiency anemia)
Anemia of Chronic Disease
-Infections, inflammation, malignancy
-Iron metabolism disturbed
-Normochromic, normocytic anemia
-Anemia usually mild
Anemia of Renal Disease
-May see some echinocytes
-Decreased erythropoietin
Anemia of Liver Disease
-Acanthocytes, targets
-Usually complicated by other factors
Aplastic Anemia
-Pancytopenia
-Empty marrow
-Most are idiopathic
Things you Must Know – Leukemia
Acute Leukemia
Acute Myeloid Leukemia
-Malignant proliferation of myeloid blasts in blood,
bone marrow
-20% cutoff for diagnosis
-Many subtypes
-Bad prognosis
AML-M0
-↑↑↑ myeloblasts
-Bland
-MPO negative
-Need markers
AML-M1
-↑↑↑ myeloblasts
-No maturation
-Auer rods
-MPO positive
AML-M2
-↑ myeloblasts
-Maturing neutrophils
-t(8;21) in some cases
AML-M3
-↑↑↑ promyelocytes
-faggot cells
-DIC
-t(15;17) in all cases
B-Cell Precursor ALL
- = B-cell lymphoblastic lymphoma
-Several sub- and sub-subtypes
-TdT+
-Rarely, Ph+!
B-Cell ALL
- = Burkitt lymphoma
-Blue cytoplasm with vacuoles
-TdT –
-t(8;14)
ALL
•
AML-M4
-↑↑ myeloblasts
-↑↑ monocytic cells
-extramedullary tumor masses
-inv(16) in some cases
•
Prognosis
- Immunophenotype (T is bad)
- Age (1-10 is good)
- WBC (<10,000 good)
- Cytogenetics (hyperdiploidy good!)
Treatment
- Chemo ± bone marrow transplant
- Many children are cured!
AML-M5
-↑↑ monocytic cells
-NSE positive
-M5A and M5B
-Extramedullary tumor masses
AML-M6
-↑↑ erythroblasts
-↑↑ myeloblasts
-Dyserythropoiesis
AML-M7
-↑↑ megakaryoblasts
-Bland blasts
-MPO negative
-Need markers
Chronic Lymphocytic Leukemia
-Small, mature lymphocytes
- = small lymphocytic lymphoma
-CD5+
-Long but inexorable course
Hairy Cell Leukemia
-Hairy cells
-Big spleen
-Pancytopenia
-TRAP
Prolymphocytic Leukemia
-Prolymphocytes
-Splenomegaly without LNy
-Rare
-Aggressive
Acute Lymphoblastic Leukemia
-Malignant proliferation of lymphoid blasts in blood,
bone marrow
-Classified by immunophenotype (B vs. T)
-More common in children
-Prognosis often good!
T-Cell ALL
- = T-cell lymphoblastic lymphoma
-Teenage male with mediastinal mass
- WBC usually ↑↑↑
-Bad prognosis
Large Granulated Lymphocyte Leukemia
-Large granulated lymphocytes
-T-cell
-Neutropenia
-Long Survival
Multiple Myeloma
- monoclonal plasma cell proliferation
- monoclonal gammopathy
- decreased normal immunoglobulins
- osteolytic lesions
Chronic Myeloproliferative Disorders (4)
-
Chronic myeloid leukemia – the main one
Polycythemia vera
Essential thrombocythemia
Chronic myelofibrosis
Features of All myeloproliferative Disorders
-
Occur only in adults
Long clinical course
↑ WBC with left shift
Hypercellular marrow
Big spleen
Chronic Myelogenous Leukemia
-
Neutrophilic leukocytosis
Basophilia
Philadelphia chromosome
Three phases
Chronic Myelofibrosis
- Panmyelosis……then marrow fibrosis
- Extramedullary hematopoiesis
- Teardrop red cells
Polycythemia Vera
-
High RBC in blood (makes blood sludgy)
Different from secondary polycythemia!
Thrombosis and hemorrhage
Jak-2 mutation
Essential Thrombocythemia
-
Very high platelet count in blood
Can occur in young women of child-bearing age.
Diagnosis of exclusion
Hemorrhage and thrombosis
Follicular Hyperplasia
-
Large, irregular follicles
Mixture of cells in germinal centers
Tangible-body macrophages
B cell response to some immune stimulus
Interfollicular Hyperplasia
- Expanded area between follicles
- Mixture of cells down there
- Partial effacement
- T cell response to some immune stimulus
Non-Hodgkin Lymphomas
- Malignant proliferation of lymphoid cells (blasts or
mature cells) in lymph nodes
- Skips around (liver, lungs, leg, abdomen)
- Many sub-types
- Most are B cell
Non-Hodgkin Lymphoma Symptoms
- Painless, firm lymphadenopathy
- Extranodal manifestations
- “B” symptoms: weight loss, night sweats, fever
Small Lymphocytic Lymphoma
- Small, mature lypmhocytes
- Same thing as CLL (now grouped together)
- CD5+ (also B cell marker)
- Long course; death from infection
Marginal Zone Lymphoma
- Actually a bunch of lymphomas
- Marginal zone pattern
- MALT lymphoma
- Helicobacter pylori
Mantle Cell Lympoma
- Mantle zone pattern
- Small, angulated lymphocytes
- T(11:14) – bcl-1 and IgH
- More aggressive (but low grade)
Follicular Lymphoma
- Follicular pattern (later, diffuse)
- Small cleaved cell, mixed, or large cell
- Grade 1,2,or 3
- Translocation btwn 14:18 – IgH and bcl-2
- (bcl-2 prevents apoptosis)
Mycosis Fungoides/Sezary Syndrome
- Skin lesions in both
- Blood involvement in Sezary
- Cerebriform lymphocyte
- T-cell immunophenotype (test question)
- (test question, T cell stuff likes to go to the skin)
Diffuse Large Cell Lymphoma
- Large B cells
- Extranodal involvement
- Grows rapidly
- Poor Prognosis
Lymphoblastic Lymphoma
- Typical patientL teenage maile with mediastinal
mass (arise outside the marrow = T-cell)
- Diffuse pattern, lyphoblasts
- Same as ALL*
- Which kinds of ALL?
Burkitt Lymphoma
- Child with fast-growing, extranodal mass
- Starry-sky pattern
- Translocation (8:14)
- Same as B-cell ALL
Adult T-cell Leukemia/Lymphoma
- Japan/Caribbean basin
- HTLV-1 [Human T-lymphytropic virus-1]
- Skin lesion, hypercalcemia
- Very aggressive
The ones listed below are Hodgkin!
Hodgkin Lympoma
- Younger patients, good prognosis
- Contiguous spread
- Five subtypes
- Reed-Sternberg cell