major conditions hematology

7/29/2019 Major Conditions hematology

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Major Conditions Immunology

Lymphocytosis

With features of lymphocytetransformation, ie, medium and largelymphocytes and plasmacytoid cells1

Active immune responses, especially in children.Immunizations; bacterial infections (pertussis); viral infections(infectious mononucleosis, mumps, measles, viral hepatitis,rubella, influenza); toxoplasmosis

Mixed T and B cell (polyclonal)

Primary neoplasms some variants of chronic lymphocytic

leukemia (CLL), lymphoma

T or B cell (monoclonal) 2

Majority resemble resting smalllymphocytes

Chronic infections (tuberculosis, syphilis, brucellosis);autoimmune diseases (myasthenia gravis); metabolic diseases(thyrotoxicosis, Addison's disease)

Mixed T and B cell (polyclonal)

Primary neoplasms CLL, some small cell lymphomas T or B cell (monoclonal) 2

Lymphocytes resemble fetallymphoblasts

Primary neoplasms acute lymphoblastic leukemia,lymphoblastic lymphoma

Nonmarking or T cell or B cell(monoclonal) 2

Admixture of abnormal lymphoidcells (rare)

Primary neoplasms involvement of blood by lymphoma ormyeloma

Abnormal cells are T or B cells(monoclonal) 2 often admixedwith residual normal cells

Lymphopenia

Deficiency of T cells or B cells (orsubsets thereof) or of both T and Bcells

Toxic drugs and chemicals; steroid therapy, Cushing's disease;early phase of marrow involvement by leukemia;immunodeficiency states


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Peripheral Blood morhoplogy Conditions comments

Normal numbers of neutrophilsWith shift to the left(less mature

Leukoerythroblastic anemia Physical replacement of BMby fibrosis/ neoplasms

Primary neoplasms: early preleukemicmyeloid leukemia

Leukemic marrow or anymyeloproliferative disorders

With shift to the

right (more mature)

Megaloblastic anemias: folate antagonists Vitamin 12 or folate levels

decreased in blood; may alsoproduce neutropenia

With abnormalgiant granulocytesor inclusions

Mucopolysaccharidosis (AlderReilly; rare)

ChdiakHigashi syndrome (rare)

Toxic granules - severe infection (common)

Neutrophil leukocytosisMainly maturesegmented forms;

mild left shift

Metabolic diseases (uremia, gout); drugs(phenacetin, digitalis); postnecrosis

(myocardial infarction, burns); postsurgery;acute infections (pyogenic cocci, Escherichiacoli, Proteus, Pseudomonas, less oftentyphus, cholera, diphtheria)

Toxic granulationGiant toxic granules (Dhle

bodies)

high proportion ofless mature cells(bands/metamyelocytes);marked left shift

Leukemoid reaction (very severe acuteinfections, especially in child)

High leukocyte alkalinephosphatase (LAP) level.

Primary neoplasms: CML; less often,polycythemia rubra vera or myelosclerosis

Low LAP level in CML

high proportion ofblasts; extreme left

shift

Primary neoplasms; AML and variants Auer rods in blast cells

NeutropeniaMay occur alone ormay accompanylymphopenia,thrombocytopenia,anemia

Variable Infections: many viral (hepatitis, measles);some rickettsial rare bacterial (typhoid fever,brucellosis); malaria; any very severeinfection (septicemia, miliary TB)

Acute leukemia Early phase

Drugs Sulfonamides, analgesics,

Marrow aplasia Including druginducedVitamin B12, folate deficiency

Autoimmune diseases; Felty's syndrome Antileukocyte antibodiesFamilial cyclic neutropenia Cyclic stem cell failure (?)

Etiologic Mechanisms and Causes of Neutropenia.

1. Decreased marrow proliferationa. Infantile neutropenia (Kostmann): a rare autosomal recessive disease manifesting with

severe neutropenia at birth.


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b. Cyclic neutropenia: usually familial, autosomal dominant with onset in childhood. Profoundneutropenia lasts 34 days and occurs in cycles of about 3 weeks.

c. Drugs that suppress granulopoiesis: anticancer drugs certain antihistamines, antithyroiddrugs, tranquilizers gold salts, diuretics, penicillins, chloramphenicol, and antituberculous

drugs.

d. Radiatione. Megaloblastic anemia (decreased DNA synthesis).f. Aplastic anemias, certain refractory anemias.g. Marrow replacement by leukemia, lymphoma, fibrosis: leukoerythroblastic anemia.

2. Reduced peripheral granulocyte survivala. Viral and rickettsial infectionb. Severe bacterial sepsisc. Drugs that cause immune destruction of granulocytes: phenylbutazone, cephalothin,

aminopyrine

d. Systemic lupus erythematosus and Felty's syndrome (immune destruction)e. Hypersplenism

3. Increased egress from circulation (pseudoneutropenia)a. Viral and rickettsial infectionsb. Histamine

4. Decreased mobilization from marrowa. Lazy leukocyte syndrome

Abnormality Appearance Disease

Toxic granulation Cytoplasmic granules become

coarse and more darkly staining

Infections and inflammatory

disease

Dhle bodies 1 to 2m blue granules in

cytoplasm

As toxic granulation, plus

myeloid leukemias (also seen in

cyclophosphamide therapy)

Auer rods 1

to 4

m red rods in blast cells Acute myeloblastic leukemiaPelgerHut anomaly Bilobed or nonsegmented

neutrophils

Hereditary; also myeloid

leukemias

MayHegglin anomaly Basophil inclusions that resemble

Dhle bodies

MayHegglin syndrome (giant

platelets and thrombocytopenia)

hronic granulomatous

disease of childhood

Xlinked

recessive

Childhood Failure to produce peroxide by neutrophils,

monocytes, leading to recurrent infections with

catalaseproducing organisms ( Staphylococcus

aureu s, Candida spp, gramnegative enteric

bacilli, Aspergillus spp)

Myeloperoxidasedeficiency

Autosomalrecessive

Asymptomatic Myeloperoxidase deficiency in neutrophils,monocytes; usually no clinical effect

Type of Leukemia Chromosomal Abnormality

Chronic myelocytic leukemia (CML) Philadelphia chromosome t(9;22)

CML in blast crisis t(9;22) + 8, 2 isochromosome of 17 or 4

Acute myeloblastic leukemia (AML) t(8;21), t(9;22) +8, 7, 5, 7q, 5q

Erythroleukemia 7q, 5q

Acute monocytic leukemia t(9;11), t(11;23)

Acute promyelocytic (M3) leukemia t(15;17)


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Polycythemia rubra vera 20q

Acute lymphoblastic leukemia (ALL) 6q, t(4;11), t(9;22), t(8;14)

Chronic lymphocytic leukemia (CLL) +12

Type Peroxidase Sudan

Black

Chloroacetate

Esterase

Nonspecific

Esterase

Periodic

Acid-

Schiff

Morphologic

Features

Lymphoblastic

(ALL) 1 + Single nucleolus

Myeloblastic

(AML)

+ + + Multiple

nucleoli, Auer

rods

Monocytic +

Myelomonocytic + + + +

Unclassified

raditional

Nomenclature

Lineage T Cell Antigens

(CD2, CD3, CD5,

CD7, CD8) 2

B Cell

Antigen

(CD19)

CALLA 3

(CD10)

Tdt4

Gene

Rearrangement

Ig TCR

Null cell Earliest

recognizable B

cell

+ + +

Common 5 Early B cell + + + +

B cell 6 Late fetal B cell + +

T cell T cell + + +


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major conditions hematology

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