M.A.S
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A 66 year old woman presents with general feelings of malaise
over the past three months. Closer questioning reveals that
over this period she has had progressive fatigue and some night
sweats. She has lost her appetite, has early satiety, occasional
vomiting and her weight is 7 kg lower than six months ago
although her clothes seem to be tighter around her waist.
Sometimes she has pain and tenderness, swelling and redness
of her right big toe. Her history is otherwise unremarkable,
although she thought that in the past a doctor had told her
that she ‘had too many red blood cells’. On physical examination
she has marked abdominal distention, with massive
splenomegaly, hepatomegaly and ascities. Initial workup
included a complete blood count which revealed anemia
(79 g/L) and thrombocytopenia (platelet count 23 x 109/L). The
peripheral blood film is shown below.
What is the likely diagnosis?
What are common management options?
What complications might this patient develop?
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| gballarino
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:? mmm?
(thank god we´ll have options in USMLE)
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Guillermo Ballarino
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| gballarino
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Well...
I´m thinking some of the myeloproliferative diseases. I would begin the workup by ordering a medullary biopsy.
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Guillermo Ballarino
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| alice8
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I am not sure but i think there are some tear drop cells (dacryocyts) in the blood smear,and with the previous history of this patient it sounds like the patient had a polycytemia vera or something like that ,precursor of something malignant like myelofibrosis with myeloid metaplasia, or chronic myeloproliferative disorder...
old lady with anemia,hypermetabolism (night sweats,weight loss etc),hepato and splenomegaly...i think bone pain is because of increased cell turnover...
so if the diagnosis of myelofibrosis is right :arrow: managment would be symptomatic and specific...like blood transfusions for anemia,allopurinol and hydration for hypermetabolism and hyperuricemia...
complication would be something because of anemia,bleeding or more severe like transformation of acute leukemia...
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| alice8
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i forgot to add hydroxyure for managment of this disorder
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| zaki
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Agree with the alice, this lady had got polycythemia vera,as suported by her Hb level, blood smear shows hypochromia and poikilocytosis whcih also suported the dx and hyperurecimea with resultant gout symptoms.
she had one of the complication of polychthemia vera( already and that is hepatic venous thrombosis, thats y she had splenomegaly and ascites sign of portal hypertension.
management options include phlebotomy 500ml/week until Hct is <45 and avoid iron supplements. plus hydroxy urea+allopurinol.
occ myelosuppressive therapy is indicated
aspirin use is controvesial.
complication as alice told is myelofibrosis,arterial thrombosis(most common cause of death in these pts),and CML.
in myelofibrosis smear show teardrop poikilocytosis and nucleatead RBCs. and pt usually presents with anemia.
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| gballarino
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guys... questions like this make me wonder...
shouldn´t I be sendint asap a form to ask for a change in my elegibility period?!?!? :cry:
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Guillermo Ballarino
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| M.A.S
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Hi everyone
zaki, are you talking about polycythemia rubra vera (PRV) or myelofibrosis - in this pt?
In case of PRV you can do phlebotomy, but you can't do that in anemic myelofibrotic patient, I think.
This old woman has myelofibrosis -alice8 is right. The source of this question is University of Toronto Medical Journal.
Myleofibrosis is closely related to polycythemia rubra vera and a quarter of patients have a previous history of that disease.
By the way, the presence of teardrop-shaped red cells, nucleated red cells, myelocytes, and promyelocytes establishes the presence of extramedullary hematopoiesis, (the marrow is fibrotic).
I would ask both of you, why to give this pt hyroxyurea - which is an antineoplastic agent and used in treatment of (e.g.) essential thrombocytosis - and the pt here has too little platelets??? anyone to explain?
Our opthalm. exam is next Mon. I will be buisy somewhat.
Regards
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actually i msiunderstant Hb level 79g/L(7.9g/dl) to Hct level of 79 .my mistake if she is anemic this is clearly myleofibrosis. my mistake
no need for hydroxy urea then
hydroxy urea use in polycythemia to reduce the chance of leukomogenc potential of alkylating agent when use in polycythemia.
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| alice8
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yes you are right about hydroxyurea..it is given to myelofibrotic patients to control excessive platelets and white blood counts ...this patient has thrombocytosis :roll: :oops:
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| alice8
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oops ,i am sorry ,i mean thrombocytopenia ,not thrombocytosis in my previous message :oops:
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| clipper79@aol.com
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My husband and I live in Florida, I am one of 6 siblings. 6 months ago my brother (36) died of CML leukemia. My mom, age 75, has told me she has myelofibrosis. My sister is the main caretaker and she is having a hard time in driving back and forth to the hospital with my mothers condition seeming to weaken drastically since the death of her son. She now has (on top of myelofibrosis) dementia, won't eat and won't shower, etc. My father (who has macular degeneration) and still suffering the loss of his son is the main caretaker. We all don't know what to do. Her psychiatrist says shock treatments, her blood Dr. gives her transfusions and her MD tries to keep up with all of this. WHAT DO WE DO?
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