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A 31-year-old female is referred to your clinic for an evaluation of anemia. She describes a
2-month history of fatigue. She denies abdominal pain but notes that her abdomen has become
slightly more distended in recent weeks. Past medical history is otherwise unremarkable. The
patient's parents are alive, and she has three healthy siblings. Physical examination is
significant for pale conjunctiva and a palpable spleen 4 cm below the left costal margin.
Hematocrit is 31% and bilirubin is normal. The reticulocyte percentage is low. Haptoglobin and
lactic dehydrogenase (LDH) are normal. A peripheral blood smear shows numerous
teardrop-shaped red cells, nucleated red cells, and occasional myelocytes. A bone marrow
aspirate is unsuccessful, but a biopsy shows a hypercellular marrow with trilineage hyperplasia
and findings consistent with the presumed diagnosis of chronic idiopathic myelofibrosis. You
transfuse her to a hematocrit of 40%.
What is the most appropriate next management step?
A. Administer erythropoietin.
B. Follow up in 6 months.
C. Institute combined-modality chemotherapy.
D. Perform HLA matching of her siblings.
E. Perform a splenectomy.
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B.?????
Treatment of myelofibrosis aims to control symptoms without causing too many side effects.
If symptoms are caused by a low red blood cell count (anaemia), one can be given transfusions of blood. Blood transfusions can be repeated as often as necessary.
Chemotherapy may be used to treat myelofibrosis, especially if the symptoms are severe. One of the side effects of chemotherapy is to temporarily reduce the function of the bone marrow. For this reason, chemotherapy is not suitable for everyone with myelofibrosis.
splenectomy:- if very low number of platelets, or if your spleen is enlarged and contains many blood cells as this will cause pain and discomfort. A splenectomy will improve symptoms. This can be a risky operation for someone with myelofibrosis,
so ans shud be B ????????????
BUT Disease-associated anemia may occasionally respond to erythropoietin....
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i think ddddd or c.... il go with d now... not an easy q..for me atleast
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hla matching for bm transplantaion...
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Patients with mild forms of the disease may require no therapy or occasional transfusion support....
since she is transfused to a hematocrit of 40%.
so according to me now Follow up is required....
if still anemia persist we can go for erythropoietin.Erythropoietin may increase red blood cell production and decrease transfusion requirements.
but i agree q is not simple......
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yes even D can be right...
as Allogeneic bone marrow transplantation has been performed successfully with 50% long-term survival and should be considered in younger patients
ans plzzzzzz
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A bone marrow aspirate is unsuccessful, but a biopsy shows a hypercellular marrow with trilineage hyperplasia and findings consistent with the presumed diagnosis of chronic idiopathic myelofibrosis..... so its severe..... now if we get a matching sibling why put patient under stressful therapy...i still go with d...plz correct me
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The answer is D.
Chronic idiopathic myelofibrosis is a clonal disorder of a multipotent hematopoietic progenitor
cell of unknown etiology that is characterized by marrow fibrosis, myeloid metaplasia,
extramedullary hematopoiesis, and splenomegaly. The peripheral blood smear reflects the
features of extramedullary hematopoiesis, with teardrop-shaped red cells, immature myeloid
cells, and abnormal platelets. Leukocytes and platelets may both be elevated. The median
survival is poor at only 5 years. These patients eventually succumb to increasing organomegaly,
infection, and possible transformation to acute leukemia. There is no specific therapy for
chronic idiopathic myelofibrosis. Erythropoietin has not been shown to be consistently effective
and may exacerbate splenomegaly. Supportive care with red blood cell transfusions is
necessary as anemia worsens. Chemotherapy has no role in changing the natural history of the
disease. Some newer agents, such as interferon and thalidomide, may play a role, but their
place is not clear. Splenectomy may be necessary in symptomatic patients with massive
splenomegaly. However, extramedullary hematopoiesis may worsen with rebound
thrombocytosis and compensatory hepatomegaly. The only potential curative modality is
allogeneic bone marrow transplantation. Morbidity and mortality are high, particularly in older
patients. In light of this patient's young age and the presence of three healthy siblings, HLA
matching of her siblings is the most reasonable step.
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