White Cells Disorders

Dr. Mehzabin Ahmed

Classification

Disorders of white blood cells can be classified into two broad

categories:

Leukocytosis (Proliferative disorders)- in which there is an

increase in numbers of leukocytes

Leukopenia - in which there is a decreased number of

leukocytes

Proliferations of white cells can be reactive or neoplastic.

White Blood Cell CountWBC = 4-10,000/mm3 (normal)

Leukopenia

This is a reduction in the number of Total WBCs less than

normal in the peripheral blood

Leukopenia could be due to Neutropenia, Lymphopenia or both

Neutropenia

reduction in number of neutrophils below normal

It may be due to:

Reduced production of neutrophils

Accelerated removal of neutrophils from the circulating blood

Altered distribution of Neutrophils (Drugs, stress)

A fall in neutrophil counts below 500/mL is called Agranulocytosis

1-Decreased Production

Suppression of myeloid stem cells, as occurs in:

Aplastic anaemia

Infiltrative marrow disorders (tumors, granulomatous disease, etc.)

Exposure to certain drugs ( Cytotoxic drugs, chemotherapy)

Ineffective granulopoiesis in disease states such as:

Megaloblastic anaemias due to vitamin B12 or Folate deficiency and

where defective precursors are susceptible to death in the marrow

Myelodysplastic syndromes.

Pure WBC aplasia

2-Accelerated removal or destruction of Neutrophils

Immunologically mediated injury to neutrophils

Associated with immunologic disorders, eg/ SLE, Feltys

syndrome

Hypersplenism: in which excessive destruction occurs secondary

to enlargement of the spleen, usually associated with increased

destruction of red cells and platelets as well.

Increased peripheral utilization -as may occur in overwhelming

bacterial, fungal, or rickettsial infections

Drug induced

Complement mediated destruction-

-Hemodialysis

-Cardiopulmonary bypass

Lymphopenia

Lymphopenia is a reduction in the Lymphocyte count below normal

Causes

Primary Lymphopenia:

Primary immunodeficiency diseases

Eg: Severe combined immunodeficiency

Secondary Lymphopenia: many causes

Infections: Influenza, Miliary Tb, Malaria, HIV

Loss of Lymphocytes: Whipples disease, severe Right side Heart failure,

Lymphatic fistula

Drugs: Radiotherapy, Corticosteroids, Cytotoxic drugs

Neoplastic conditions: Metastatic CA, advanced Hodgkin’s disease

Nutritional/Metabolic: B12/ Folate deficiency, Uremia

Others: SLE, Aplastic anemia, Graft v Host disease, Sarcoidosis, Idiopathic

Neutrophilic Leucocytosis

Neutrophilia is the increase in the Neutrophil count above 7.5×109

Causes of Neutrophilia

1-Bacterial infections: Pyogenic infections

2-Inflammation and Tissue necrosis: cardiac infarction, trauma, vasculitis

3-Metabolic disorders: uremia, eclampsia, acidosis, gout

4-Neoplasms: CA, Lymphoma, Melanoma

5-Acute hemorrhage or hemolysis

6-Corticosteroid therapy (inhibits margination)

7-Myeloproliferative diseases: CML, PRV

8-Treatment with myeloid growth factors: G-CSF, GM-CSF

White Blood Cells

Leucocytosis - WBC count

Granulocytosis

Lymphocytosis

Monocytosis

Eosinophilia,

Basophilia

Leukopenia - WBC count

Neutropenia,

Lymphopenia

Agranulocytosis - 500 mm3

Granulocytosis (Neutrophilia)

Neutrophilia may be

accompanied by fever due

to the release of Leukocyte

pyrogens

Reactive Neutrophilia

Characteristic features of Reactive Neutrophilia

Shift to the left in the peripheral blood differential WBC count

(increase in the number of band forms, myelocytes,

metamyelocytes)

1.Toxic granulations- coarse dark cytoplasmic granules2. Dohle Bodies- blue cytoplasmic patches of dilated endoplasmic reticulum (arrow)

Reactive changes in neutrophils

Toxic granulations Dohle Bodies

Activated neutrophils show increased alkaline phosphatase activity and give rise to Increased NAP scores

Lymphocytosis - causes

Most often viral infection

Chronic inflammation

Marked lymphocytosis with

activated lymphocytes – seen in

infectious mononucleosis (EBV)

Lymphocytes - NormalLymphocytes - Normal Lymphocytosis

Monocytosis

Causes of Monocytosis include:

Chronic bacterial infections -bacterial

endocarditis, malaria, Tb, Typhoid Chronic inflammation- Collagen

vascular diseases, SLE, RA, ulcerative

colitis, Sarcoidosis Malignant- AML, CA, MDS, Hodgkins

disease Others- post splenectomy, chronic

neutropaenia

Eosinophilia

Causes of Eosinophilia include:

Allergic disorders: hay fever, Bronchial asthma, urticaria, food

hypersensitivity

Skin disease: Psoriasis, Pemphigus

Parasitic infestations: Amoebiasis, Ascariasis, Hookworm,

Filariasis

Pulmonary eosinophilia and the Hypereosinophilic syndrome

Drug sensitivity

Hodgkins disease

Metastatic malignancy with tumor necrosis

Eosinophilic leukaemia

Basophilia

Basophilia occurs in Myeloproliferative disorders (CML)

Leukaemoid Reaction

This is a reactive and excessive leucocytosis, characterized by: WBC counts up to 15 - 100,000/mm3

Shift to the left (immature WBCs) Signs of activation Dohle bodies, toxic granulations (Differs

the condition from CML) NAP scores high (Differs the condition from CML) MPO negative (Myeloperoxidase) - reverse of CML Underlying disease:

Severe or chronic infections

Severe haemolysis

Metastatic CA Leukamoid reactions are particularly marked in children

Leukaemoid Reaction

LAP +veMPO +vein CML

“Shift to the Left”

LEUKAEMIA

it is the malignant proliferation of the WBC, with the presence of the immature

forms in the peripheral circulation

MYELOMA

it is the malignant proliferation of the plasma cells, the immunoglobulin

producing cells.

Malignancies of the leukocytes

LEUKEMIA

Definition

Leukemia is a type of cancer. It is the cancer of the blood cells.

Leukemia

When leukemia develops, the body produces large numbers of

abnormal blood cells.

In most types of leukemia, the abnormal cells are white blood

cells.

The leukemia cells usually look different from normal blood cells,

and they do not function properly.

Causes of leukemia Leukemia occurs more often in males than in females and In white people more often than in black people. Certain risk factors increase a person's chance of developing

leukemia. For example, Exposure to large amounts of high-energy radiation. Exposure to electromagnetic fields is a possible risk factor

(Electromagnetic fields are a type of low-energy radiation that comes from power lines and electric appliances.)

Certain genetic conditions can increase the risk for leukemia. One such condition is Down's syndrome. Children born with this syndrome are more likely to get leukemia than other children.

Workers exposed to certain chemicals over a long period of time. Benzene is one of these chemicals.

Anti cancer drugs

Types of leukemia

There are several types of leukemia.

They are grouped in two ways.

Acute vs Chronic

Lymphoid vs Myeloid

ACUTE & CHRONIC

Based on how quickly the disease develops and gets worse

In acute leukemia,

the abnormal blood cells are blasts that remain very immature

cannot carry out their normal functions.

The number of blasts increases rapidly, and

the disease becomes worse quickly.

In chronic leukemia,

some blast cells are present, but in general, these cells are more mature

can carry out some of their normal functions.

the number of blasts increases less rapidly than in acute leukemia, thus

chronic leukemia worsens gradually.

LYMPHOID & MYELOID Based on the type of blood cell that is affected. lymphoid cells, - lymphocytic leukemia. myeloid cells - myeloid or myelogenous leukemia. These are the most common types of leukemia:

Acute lymphocytic leukemia (ALL) is the most common type of leukemia in young children. This disease also affects adults, especially those age 65 and older.

Acute myeloid leukemia (AML) occurs in both adults and children. This type of leukemia is sometimes called acute non-lymphocytic

leukemia (ANLL). Chronic lymphocytic leukemia (CLL)

most often affects adults over the age of 55. It sometimes occurs in younger adults, but it almost never affects

children. Chronic myeloid leukemia (CML)

occurs mainly in adults. A very small number of children also develop this disease.

Symptoms of leukemia Infections and fevers as leukemia cells are abnormal cells that cannot

help the body fight infections.

People with leukemia often have anemia and there are not enough

red blood cells to carry oxygen through the body. The patients look

pale and feel weak and tired.

A decreased count of platelets (thrombocytopenia), causes the

patients to bleed and bruise easily.

In acute leukemia, symptoms appear and worsen quickly.

In chronic leukemia, symptoms may not appear for a long time. When

symptoms do appear, they generally are mild at first and get worse

gradually.

These are some of the common symptoms of leukemia:

Fever, chills, and other flu-like symptoms;

Frequent infections;

Swollen or tender lymph nodes, liver, or spleen;

Weakness and fatigue;

Loss of appetite and/or weight;

Easy bleeding or bruising;

Tiny red spots (called petechiae) under the skin;

Swollen or bleeding gums;

Sweating, especially at night; and/or

Bone or joint pain.

Symptoms of leukemia (contd)

In acute leukemia The abnormal cells can collect in the brain or spinal cord (also

called the central nervous system or CNS). headaches, vomiting, confusion, loss of muscle control, and

seizures. Leukemia cells also can collect in the testicles and cause swelling. Some patients develop sores in the eyes or on the skin. Leukemia also can affect the digestive tract, kidneys, lungs, or

other parts of the body. In chronic leukemia

The abnormal blood cells may gradually collect in various parts of the body.

Chronic leukemia can affect the skin, central nervous system, digestive tract, kidneys, and testicles.

WBC Disorders

At the end of the lesson on WBC Disorders, the student should be able to:

Define terms – Leucocytosis, Leucopenia, leukemia, myeloma.

Enumerate the common causes of neutrophilia, eosinophilia & lymphocytosis.

Classify leukemia. List the clinical manifestation of acute

leukemia