A 13-year-old boy is evaluated for isolated thrombocytopenia.
The first step in his evaluation is
- Bone marrow aspiration and biopsy
- Peripheral blood smear
- Anti-platelet antibody titres
- Clinical examination for splenomegaly
Answer: The answer is (d). The presence of splenomegaly is extremely rare in immune thrombocytopenic purpura. This examination would give a clue to the diagnosis. A peripheral blood smear is also important to look into the morphology of the platelets and to look into the morphology of the other cells. Antiplatelet antibody titres are not sensitive or specific and hence are not helpful. A bone marrow aspiration and biopsy is indicated in certain specific cases where a bone marrow pathology might be the cause of the disease.
The diagnosis of immune thrombocytopenia is made. The treatment includes:
- Glucocorticoids with addition of intravenous immunoglobulin if actively bleeding
- Non steroidal anti inflammatory agents
- Plasmapheresis
- Chemotherapy
Answer: The answer is (a). Glucocorticoids with addition of intravenous immunoglobulins if actively bleeding are the treatment of choice for immune thrombocytopenia. Non steroidal anti inflammatory agents can aggravate the bleeding in these patients are these agents inhibit platelet function. Plasmapheresis is indicated only in life threatening situations.
The boy is refractory to a course of steroids. Further management includes:
- Immunosuppression with vincristine and cyclophosphamide.
- Intravenous immunoglobulins
- Anti-D immunoglobulin injections
- Splenectomy
Answer: The answer is (d). Splenectomy is the treatment of choice in refractory cases of immune thrombocytopenia. It can salvage almost two thirds of all refractory cases of immune thrombocytopenia.
If a splenectomy were planned for him, which of the following vaccines would not be indicated?
- Hemophilus influenzae type B
- Influenza
- Pneumococcal
- Neisseria meningitidis vaccine.
Answer: The answer is (b). Spleen is the site for destruction of encapsulated bacteria. Therefore Hemophilus influenzae, Streptococcus pneumoniae and Neisseria meningitidis are organisms, which cause recurrent infections in splenectomized patients. Patients who are scheduled for splenectomy need to receive vaccines against these organisms at least 2 weeks prior to surgery.