Aplastic Ad Plycythemia

Question 1

The main causes of aplastic anemia are:

Answer 1

The main causes of aplastic anemia are:

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Idiopathic (in above 65% of cases).

Chemicals (benzene and related compounds).

Drugs ( chloramphenicol, gold salts, D-peniciline, Cytotoxic drugs ,Immunosuppressives-as azathioprine Radiation.

Viruses (hepatitis C, parvovirus B19, Epstein-Barr virus, cytomegalovirus). Congenital (Fanconi anemia).

Other (connective tissue disease(SLE),pregnancy, Paroxysmal nocturnal hemoglobinuria(PNH.)

Question 2

Fanconi’s anemia is inherited as

Answer 2

autosomal recessive trait.

Question 3

Physical Abnormalities in Fanconi’s Anemia

Answer 3

Skin hyperpigmentation , Short stature, Hypogonadism and genital abnormalities (males), Other skeletal. Anomalies of eyes, eyelids. Renal abnormalities.

Question 4

Camitta criteria

Answer 4

Sever Aa
Marrow cellularity less than 25% and haemophilic stem less than 30% and two of the below
Neutrophil is less than 0.5 *10 ‘o
Platelet less than 20 *10’9
Reiculocyte like platelet

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Very severe as above but neutrophils less than 0,2
..
Non severe not fulfil the criteria of the SAA or VSAA

Question 5

2.Definitive Therapy:

Answer 5

Immunosuppressive therapy includes antithymocyte globulin (ATG) and cyclosporine. A definitive therapy for aplastic anemia include bone marrow transplantation and immunosuppressive therapy. Bone marrow transplantation is considered the treatment of choice in young, otherwise healthy patients with severe aplastic anemia who have a related histocompatible donor.

Question 6

1.Supportive care for AA

Answer 6

includes red cell transfusions for symptomatic anemia and platelet transfusions for bleeding due to thrombocytopenia. Prophylactic platelet transfusions should be considered for patients with severe thrombocytopenia (5,000–10,000) even in the absence of bleeding. It is desirable to use single donor platelets as much as possible to avoid alloimmunization and the patient becoming refractory to future platelet transfusions.

Transfusions from relatives or potential bone marrow donors should be avoided to decrease the chance of later marrow transplant rejection.

Antibiotics should also be given for fever or infection in the presence of neutropenia (absolute neutrophil count 500–1,000). Initially, broad-spectrum antibiotics should be used for fever, with specific antibiotics chosen based on results of the cultures.

Question 7

Polycythemia and it’s cause

Answer 7

hemoglobin level greater than the upper limit of normal (adult females 165 g/L or hematocrit > 0.48; adult males 180 g/L or hematocrit > 0.52) for more than 2 months should be investigated.

may be due to an increase in the number of red blood cells (true polycythemia) or a reduction in the plasma volume (relative or apparent polycythemia.

Question 8

Classification of polycythemia :.
And management

Answer 8

Primary polycythemia (POLYCYTHEMIA RUBRA VERA):

It is a rare blood disorder characterized by the overproduction of red blood cells (erythrocytes) in the bone marrow due to JAK 2 gene mutation characterized by splenomegaly and itchy skin after hot shower. It will be covered in the lecture on myeloproliferative disorders.

Secondary polycythemia: True polycythemia and Relative polycythemia

Unlike polycythemia vera, which is a primary disorder arising from genetic mutations in bone marrow cells, secondary polycythemia is a reactive response to an underlying cause or condition.

Due to secondary causes most commonly are COPD , Hypoxia and congenital heart diseases.

Management :

No medications are available to treat secondary polycythemia. Treat the underlying causes.

Provide oxygen supplementation to patients with chronic obstructive pulmonary disease. Recommend weight loss in patients with obesity and hypoventilation.

Recommend smoking cessation for patients with carboxyhemoglobin.

Surgically correct arteriovenous shunts.

To restore viscosity and maintain circulation at its optimal level, phlebotomy to ameliorate the symptoms of polycythemia and also decrease the risk of thrombosis, strokes, myocardial infarction, and DVT.

Question 9

Gaisböck’s syndrome

Answer 9

Apparent erythrocytosis with a raised Hct, normal red cell mass (RCM) and reduced plasma volume may be associated with hypertension, smoking, alcohol and diuretic use which is called

Question 10

CLINICAL FEATURES OF POLYCYTHEMIA:

Answer 10

A detail history and thoroughly examination will provide clues as to the etiology of polycythemia . decreased mentation, headache dizziness ,fatigue, generalized weakness, and poor exercise tolerance . plethora or a ruddy complexion .

The cardiovascular and respiratory systems should be assessed for evidence and causes of hypoxemia The presence of splenomegaly supports a diagnosis of polycythemia Vera rather than secondary polycythemia.

Cardiac murmurs and clubbing of the fingers may suggest a congenital heart disease.

Those with PRV may have arterial thromboses, pruritus worse after a hot bath, hepatosplenomegaly and gout (due to high red cell turnover) . If no other cause is identified, further investigations to exclude inappropriate erythropoietin secretion should be performed.

A clinical history and examination will identify most patients with polycythemia secondary to hypoxia.