Leukocyte Disorders

Neutrophils moves to tissues

A shift to the left occurs when:

Neutrophilic Leukemoid Reaction

Blood picture mimics chronic myelogenous leukemia (CML)

Leukoerythroblastic Reaction

Presence of immature leukocytes and immature erythrocytes in the blood

• Occurs in marrow replacement disorders such as myelofibrosis

Chronic Granulomatous Disease (CGD)

Morphologically normal, but functionally abnormal because of enzyme deficiency that results in an inability to degranulate which causes inhibited bactericidal function

Hypersegmentation

5 or more lobes in the neutrophil

Pelger-Huet anomaly

Nucleus has a dumbbell or peanut-shape; “pince-nez” appearance

• Caused by a mutation of the LAMIN B receptor

Pseudo Pelger-Huet anomaly

Acquired abnormality associated with myeloproliferative disorders and myelodysplastic syndromes; can also be drug induced

• Nucleus is usually round instead of dumbbell shape

May-Hegglin Anomaly

Large crystalline, Dohle-like inclusions in the cytoplasm of neutrophils on Wright’s stain

• Gray blue spindle (cigar) shaped

Gaucher disease

Most common lipid storage disorder

• Crumpled tissue paper appearance of macrophage

Niemann-Pick Disease

Deficiency in sphingomyelinase causes sphingomyelin to accumulate in macrophages in multiple organs and bone marrow

• Foamy appearance of macrophages (foam cells)

Fabry’s disease

A deficiency in Alpha galactosidase indicates which lipid storage disease?

Krabbe disease

A deficiency in Cerebroside beta galactosidase indicates which lipid storage disease?

Metachromatic leukodystrophy

A deficiency in Arylsulfatase A indicates which lipid storage disease?

Cytomegalovirus

It is the most common transmitted infection from mother to fetus

Infectious lymphocytosis

Associated with adenovirus and coxsackie A virus

• Contagious disease mostly affecting young children

French-American-British (FAB) Classification

Defines acute leukemia as >30% bone marrow blasts

WHO Classification

Defines acute leukemia as >20% bone marrow blasts

• Standard for diagnosis

Perl’s prussian blue

Cytochemical stain which stains siderotic granules, Pappenheimer bodies and hemosiderin

LAP

Cytochemical stain that stains ALP present in the neutrophil

Myeloperoxidase stain

Cytochemical stain used to differentiate acute myelogenous leukemia and monocytic leukemia from acute lymphocytic leukemia

• Stains peroxidases present in granulocytes and monocytes

Sudan black B

Stains lipids present in granulocytes and monocytes

• Used to differentiate acute myelogenous leukemia and myelomonocytic leukemias from acute lymphocytic leukemia

Periodic Acid Schiff

Stains mucoproteins, glycoproteins, and high molecular weight carbohydrates

• Used to help in the diagnosis of DiGuglielmo’s syndrome (FAB M6)

Naphthol AS-D Choroacetate Esterase

Stains esterases in granulocytes and mast cell granules

Toluidine blue

Binds with acid mucopolysaccharides in blood cells

• Useful for the recognition of mast cells and tissue basophils

Nitroblue Tetrazolium Test

Screening procedure for the detection of chronic granulomatous disease

FAB L1

Most common childhood ALL

FAB L2

Most common ALL in adults

FAB M3

AML classification associated with DIC

CML

It is the only chronic myeloproliferative disease that is negative for the JAK2 V617F gene

Type of chronic leukemia where platelets can reach up to >1000 x10^9/L

• JAK2 V617F (+)

• CD123

• Annexin A1

The most specific markers for classic hairy cell leukemia

Multiple myeloma

Condition in which there is presence of BJP in the urine and precipitates at 40-60C and dissolves at 100C

Waldenstroms

Lymphoplasmacytic infiltration of the B.M.

Mycosis fungoides

The most common cutaneous lymphoma

Sezary cells

What type of cells are present in Mycosis fungoides?