WBR0950

Author	[[PageAuthor::Mugilan Poongkunran M.B.B.S [1]]]
Exam Type	ExamType::USMLE Step 3
Main Category	MainCategory::Primary Care Office
Sub Category	SubCategory::Hepatology, SubCategory::Hematology
Prompt	[[Prompt::A 25 year old male, comes to the office with complaints of severe fatigue and lethargy for the past 1 year. On further review of history, he says he has intermittent mild abdominal pain over the past few months. Of late he is having difficulty in writing his thesis and really worried about not finishing his project on time. He says he does not talk with friends anymore and spends most of his time at home. His past history is otherwise insignificant and his family history is unremarkable. He does not smoke and drink. His vitals are temperature: 36.7 C, blood pressure: 130/70 mmHg, pulse: 72/min and respirations: 16/min. Abdominal examination reveals mild abdominal pain on the right upper quadrant and mild hepatomegaly. Cardiovascular and respiratory system examinations are normal. Neurological examinations shows mild rigidity in the upper limbs. His eyes shows a brownish discoloration of iris and skin are normal. You admit the patient and send blood sample for CBC, peripheral smear, LFT’s and electrolytes. What is the most likely peripheral smear picture you see in this patient?]]
Answer A	AnswerA::Teardrop RBC's with circulating nucleated RBC
Answer A Explanation	[[AnswerAExp:: Incorrect  : Teardrop RBCs with circulating nucleated RBC and early white blood cell forms indicate the presence of marrow involvement, as in primary myelofibrosis or tumor infiltration.]]
Answer B	AnswerB::Macrocytic RBC's with multifragmented neutrophils
Answer B Explanation	[[AnswerBExp:: Incorrect  : Macrocytic RBCs with multifragmented neutrophils is seen in vitamin B12 or folic acid deficiency patients.]]
Answer C	AnswerC::Microcytic and hypochromic RBC’s with Heinz inclusion bodies
Answer C Explanation	[[AnswerCExp:: Incorrect  : Microcytic and hypochromic RBC’s with Heinz inclusion bodies is characteristic of thalassemia.]]
Answer D	AnswerD::Spherocytes , microspherocytes, and elliptocytes
Answer D Explanation	AnswerDExp::''' Correct ''' : Spherocytes , microspherocytes, and elliptocytes are seen in autoimmune hemolytic conditions which is associated with Wilson’s disease.
Answer E	AnswerE::RBC’s with blister or bite cells
Answer E Explanation	[[AnswerEExp:: Incorrect  : Blister or "bite" cells are due to oxidant-induced damage to the red cell and its membrane and is seen in glucose-6-phosphate dehydrogenase deficiency patients.]]
Right Answer	RightAnswer::D
Explanation	[[Explanation::Wilson's disease or hepatolenticular degeneration is an autosomal recessive genetic disorder in which copper accumulates in tissues; this manifests as neurological or psychiatric symptoms and liver disease. It is treated with medication that reduces copper absorption or removes the excess copper from the body, but occasionally a liver transplant is required. The condition is due to mutations in the Wilson disease protein (ATP7B) gene. Liver disease may present as tiredness, increased bleeding tendency or confusion (due to hepatic encephalopathy) and portal hypertension. Most patients initially have mild cognitive deterioration and clumsiness, as well as changes in behavior. Specific neurological symptoms then follow, often in the form of parkinsonism (increased rigidity and slowing of routine movements) with or without a typical hand tremor, ataxia (lack of coordination) or dystonia (twisting and repetitive movements of part of the body). Seizures and migraine appear to be more common in Wilson's disease. Psychiatric problems due to Wilson's disease may include behavioral changes, depression, anxiety and psychosis. Kayser-Fleischer rings, renal tubular acidosis, cardiomyopathy and hypoparathyroidism are other manifestations. While not a common finding in isolation, Coombs-negative hemolytic anemia may be the initial symptom of Wilson disease. The anemia may occur as a single acute episode, or it may be low-grade and chronic. Educational Objective: References: ]]
Approved	Approved::Yes
Keyword	WBRKeyword::Wilson's disease, WBRKeyword::autoimmune hemolytic anemia
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