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Hematology

Question
34 out of 34
 

A patient with Myeloproliferative syndrome presents with decreased while cell count and decreased platelets The most likely diagnosis is: (AIIMS May 09)



A Chronic myeloid leukemia
B Myelofibrosis

C Polycythemias vera
D Essential thrombocytosis

Ans. B

Myelofibrosis

Myelofibrosis

1. It is a chronic myeloproliferative disorder.

2. It is characterized by splenomegaly, immature granulocytes and erythrocytes in the blood, distorted tear-drop-shaped RBC forms, and marrow fibrosis.

3. The disease is a monoclonal stem cell disease of primitive hematopoietic stem cells.

4. When fibrosis is due to a primary hematologic process, it is called myelofibrosis. When the fibrosis is secondary to a tumor or a granulomatous process, it is called myelophthisis.

Table - Disorders Causing Myelofibrosis (Ref. Hari- 18th Pg. 901, table 108.3)

Malignant

Acute leukemia (lymphocytic, myelogenous, megakaryocytic)

Chronic myelogenous leukemia

Hairy cell leukemia

Hodgkin disease

Idiopathic myelofibrosis

Lymphoma

Multiple myeloma

Myelodysplasia

Polycythemia vera

Nonmalignant

HIV infection

Hyperparathyroidism

Renal osteodystrophy

SLE

Tuberculosis

Vitamin D deficiency

The fibrosis is a secondary event.

1. Anemia and signs and symptoms of massive splenomegaly are the hallmarks of the disease.

2. Most therapy is supportive. In selected case of true hypersplenism and symptoms from massive splenomegaly, splenectomy is beneficial.

3. ANA +ve, RA +ve, Coombs +ve Hemolytic anemia (Harrison 18th -901)

4. CD34 + cell.

Triad

1. Tear drop RBC

2. Giant platelet

3. Leukoerythroblastic picture

Investigations

1. Blood film shows macrocytic anemia, leukoerythroblastic picture, tear drop poikilocytes

2. Neutrophil alkaline phosphatase score Q is high

3. Raised urate levels Q

4. Bone marrow biopsy shows an excess of megakaryocytes and increased reticulin and fibrous tissue replacement.

Diagnostic Criteria

1. Splenomegaly (may be huge)

2. Anemia (hemolytic and decreased production components)

3. Leukocytosis or thrombocytosis may be seen in 60% of patients

4. Leukoerythroblastic peripheral blood picture

5. Tailed (dacryocytes) cells on blood smear Q (Tear drop)

6. Bone marrow fibrosis (reticulin), which may be extensive

7. Osteosclerosis seen on skeletal X – rays.

Tear drop cell also seen in:

1. Myelo Dysplastic syndrome

2. Sideroblastic anemia

3. BM infiltration

4. Pernicious, IDA, thalassemia

Management

1. Blood transfusion

2. Folic acid (50 mg/day)

3. Androgen therapy (oxymetholone 50 mg/day)

4. Corticosteroids (prednisolone 40 mg/day)

5. Cytotoxic drugs (hydroxyurea upto 2 gm/day)

6. Splenectomy (in mechanical embarrassment due to massive splenomegaly, severe hemolysis, hypermetabolism, painful splenomegaly, hypersplenism).

Newer Treatment:

1. Thalidomide has produced definite responses.

2. Allogeneic bone marrow transplantation.

Hematology Flashcard List

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