Disorder of WBC

Question 1

List DDX of leukocytosis

Answer 1

Primary

1) myeloproliferative disorders - CML, polycythemia vera
2) Hereditary neutrophilia
3) familial myeloproliferative disease
4) chronic idiopathic neutrophilia

Secondary

1) infection
2) tissue necrosis - burns, cancer, infarctions
3) metabolic disorders - DKA, thyrotoxicosis, uremia
4) non haemo malignant disease
5) stress
6) Drugs - epi and steroids

Lymphocytosis
1) Viral infection
2 )Lymphoproliferative: ALL, CLL
3) Immunological response: immunizations, autoimmune disease, graft rejection

Question 2

Describe CML

Answer 2

Accounts for 15-20% of adult leukemias
Usually older than 40 and have WBC greater than 50000. THe diff count shows elevated PMN and metamyelocytes

CML is a term cell disorder in which the WBC count is elevated and the differential count is normal- platelets may be increased but RBC are down resulting in aneamia

IX - decreased leukocyte alkaline phosphatase and increased B12 with philadelphia chromosome

Question 3

Describe ALL

Answer 3

ALL is most commonly diagnosed in children younger than 10 years old. It is the most frequent malignant neoplasm in children younger than 15

The most common presenting features are nonspecific and incldue fever, bleeding bone pain and lymphadenopathy. Potential for leukostasis increases in ALL when the blast count rises above 50

Current 5 year survival is 78-85%. This response to treatment has not been found to the same degree in adults.

Question 4

Describe CLL

Answer 4

CLL is primarily a B cell disorder and is the most common type of leukaemia in the population 50 years or older.

CLL present with complaints of fatigue, weight loss and increased susceptibility to infection, rashes and easy bruising. The lymph nodes are non tender and smooth and they may appear only one or two areas. Splenic and hepatic enlargement occurs in more than 50% of patients.

Lab support of the diagnosis is

• absolute lymphocyte count >50.
• Anaemia, thrombocytopenia and neutropenia are often found.

Early therapy is directed towards complications of anaemia , thrombocytopenia, impaired or accenturated immune response or enlarged lymph nodes or spleen. Leukostasis is seldom seen in CLL but therapy is consired when counts reach 200.

Question 5

List causes of lymphopenia

Answer 5

PROLIFERATION in the BONE MARROW

1) aplastic anaemia
2) Leukamiea
3) CHemo
4) Infections - viral, T, sepsis

Maturation in the Bone marrow

1) folate or B12 defieicny, chronic idiopathic neutropenia,
2) starvation

DISTRIBUTION
1) hypersplenism: sarcoid, portal htn, malaria

Increased Use

1) infection - viral, overwhelming bacterial infection
2) autoimmune: SLE, AIDS, Felty’s syndrome

Question 6

Describe multiple myeloma

Answer 6

Plasma cell tumour
Overproduction of
-immunoglobulins of all classes
-Bence jones proteins - free kapp or lambda light chains

Feature

1) Haem
- normocytic anaemia
- thrombocytopenia
- hyperviscosity
- immune suppression and recurrent bacterial infection
- amyloidosis
2) Renal impairment
- metastatic renal clacification
- volume depletion from hypercalcaemia
- ureteric obstruction
3) Mets

Question 7

Discuss ix of multiple myeloma

Answer 7

1) Increased total protein
2) Decreased or normal albumin
3 )Serum protein electrophoresis
-increased gamma globulin
-presence of monoclonal band in 80%
-light chains in the blood only if severe renal impiarment present
4) hypercalcaemia
5) increased LDH
6) high urate
7) decreased anion gap - due to increased serum protein
8) renal impairement
9) proteinuria - bence jones protein may not be detected on bedside urinalysis
10) marked eleavtion in ESR

XR- bone marrow lesions

Bone marroy biopsy gold stanard

Question 8

Discuss management of MM

Answer 8

`General supportive

• maintain ambulation
• treat localised symptomatic lesions with radiotherapy and or surgery
• allopurinol for gout
• prednisolone for hypercalacemia
• bisphosphanates may reduce risk of path fracture and hypercalcaemia in MM