WBR0959

Author	[[PageAuthor::Mugilan Poongkunran M.B.B.S [1]]]
Exam Type	ExamType::USMLE Step 3
Main Category	MainCategory::Emergency Room
Sub Category	SubCategory::Hematology, SubCategory::Oncology
Prompt	[[Prompt::A 65 year old female comes to the emergency department with sudden onset on confusion, blurry vision and mucosal bleeding by her family members. She was in her usual state this morning but suddenly developed these symptoms. On further review of history, her family members say that she had a hip fracture 6 months back and unable to do day to day activities. She attained menopause at the age of 48 and not on any estrogen pills. Her past history is otherwise insignificant and her family history is unremarkable. Her vitals are temperature: 36.7 C, blood pressure: 140/80 mmHg, pulse: 80/min and respiration: 15/min. All system examinations are normal. The patient is treated symptomatically with I.V fluids and her laboratory values comes as : Blood glucose : 112 g/dl Hb : 10 g/dl RBC’s : 2.5 million/cmm WBC’s : 6000/cmm Neutrophils : 60 % Eosinophils : 2 % Lymphocytes : 32 % Monocytes : 6 % MCHC : 34 % MCV : 85 fl ESR : 15mm/hr Serum creatinine : 2.3 mg/dl Serum calcium : 11.7 g/dl Bone marrow biopsy done to evaluate her anemia reveals 20% plasma cells. What is the most likely cause of the patient’s condition ?]]
Answer A	AnswerA::Hypercalcemic syndrome
Answer A Explanation	AnswerAExp::''' Incorrect ''' : Hypercalcemic syndrome can cause neurological symptoms as seen in this patient, but is not associated with mucosal bleeding.
Answer B	AnswerB::Disseminated intravascular coagulation
Answer B Explanation	[[AnswerBExp:: Incorrect  : The diagnosis of DIC is suggested by the history (eg, sepsis, trauma, malignancy), clinical presentation of bleeding and thrombosis, moderate to severe thrombocytopenia (<100,000/microL) and the presence of microangiopathic changes on the peripheral blood smear. The diagnosis is confirmed by the evidence of both increased thrombin generation (eg, decreased fibrinogen, prolonged PT and aPTT) as well as increased fibrinolysis (eg, elevated FDPs and D-dimer). DIC is unlikely in this patient.]]
Answer C	AnswerC::Hyperviscoisity syndrome
Answer C Explanation	[[AnswerCExp:: Correct  : In this patient the symptoms are as a result of increased amounts of plasma cells, as seen in Waldenstrom macroglobulinemia and in certain cases of multiple myeloma in which abnormal polymers of IgA, IgG, or kappa light chains are produced.]]
Answer D	AnswerD::Acute renal failure
Answer D Explanation	[[AnswerDExp:: Incorrect  : Acute renal failure can present with neurological symptoms and bleeding tendency, but the onset is gradual and will be associated with other symptoms like edema and urinary abnormalities.]]
Answer E	AnswerE::Chronic renal failure
Answer E Explanation	[[AnswerEExp:: Incorrect  : Chronic renal failure is highly unlikely in this patient. CRF begins with damage to the nephrons, the filtering units of the kidneys, due to diseases such as diabetes, hypertension, immune complex deposition, toxin exposure, and inflammation. To compensate for the decreased glomerular filtration rate by the damaged nephrons, healthy nephrons hypertrophy and start hyperfiltrating due to signals from the body. As the disease process progresses, this adaptive response becomes maladaptive, and the increased filtration pressure in the healthy nephrons leads to the distortion of its structural architecture, causing sclerosis and eventual dropout of these nephrons.]]
Right Answer	RightAnswer::C
Explanation	[[Explanation::Hyperviscosity syndrome is an increase in the viscosity of the blood. This may be caused by an increase in serum proteins and especially monoclonal gammopathies such as Waldenström macroglobulinemia and in multiple myeloma. An increased viscosity secondary to polycythemia may be associated with organ congestion and decreased capillary perfusion. Hyperviscosity can lead to impairment in the microcirculation of the central nervous system, possibly resulting in headache, dizziness, vertigo, nystagmus, hearing loss, visual impairment, somnolence, coma, and seizures. Other possible findings include mucosal hemorrhage due to reduced platelet function, heart failure, which has been attributed to an expanded plasma volume, renal failure, and sausage-like beading in the retinal veins. The diagnosis is established by measuring serum viscosity (normal serum viscosity is between 1.4 and 1.8). Symptoms occur in most patients with values between 5 and 8. Patients presenting with severe neurologic impairment, should be treated with plasma exchange (plasmapheresis) on an emergency basis. Since plasmapheresis does not affect the disease process, chemotherapy directed against the underlying disease should be instituted at the same time. Educational Objective: References: ]]
Approved	Approved::Yes
Keyword	WBRKeyword::Multiple myeloma, WBRKeyword::Waldenstrom macroglobulinemia
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