Microangiopathic Hemolytic Anemia and Thrombocytopenia

Part 1: Diagnosis - Hematology Expert Review Questions


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Syed A. Abutalib, MD

Jean M. Connors, MD

The ASCO Post is pleased to present Hematology Expert Review, an occasional feature that includes a case report detailing a particular hematologic condition followed by questions. Answers to each question with expert commentary can be found in the sidebar. In this installment, we present the case of a 26-year old woman who presented postpartum day 8 with heavy vaginal bleeding, dizziness, and other symptoms. “Part 2: Management” will appear in the ­August 10 issue of The ASCO Post.

 

Case Study: A 26-year-old woman presented to labor and delivery triage with fatigue, heavy vaginal bleeding, and gastrointestinal (GI) upset on postpartum day 8 after vacuum-assisted vaginal delivery. Prior to presentation, she reported diarrhea and nausea with nonbilious, nonbloody emesis, which since has resolved. She had left upper quadrant pain, which worsened with deep inspiration.

On review of systems, she experienced dizziness, with no syncopal episodes. She denied fever or chills, bleeding gums or easy bruising, dysuria, or vaginal discharge other than blood.

Her medical history was notable for sickle cell trait, scoliosis, and chronic borderline hypertension. Her hypertension worsened during her most recent pregnancy, but her blood pressure normalized during the postpartum period. Her family history was notable for a mother with diabetes and hypertension and no known history of bleeding disorders.

On physical examination, her temperature was 99.4˚ F. Her heart rate was 122 beats/min, and her blood pressure ranged from 90 to 130/50 to 60 mm Hg. She was breathing at a rate of 30 to 33 breaths/min, with an oxygen saturation of 100% in ambient air. She had conjunctival pallor, and her mucous membranes were pale. Her neck was supple without lymphadenopathy, and her abdomen was soft and nondistended, with diffuse mild tenderness to deep palpation. Her lungs were clear to auscultation bilaterally. Her neurologic exam was within normal limits.

Her laboratory results revealed sodium 137 mmol/L, potassium 3.7 mmol/L, chloride 103 mmol/L, bicarbonate 20 mmol/L, calcium 8.4 mg/dL, urea nitrogen 33 mg/dL, creatinine 1.61 mg/dL (baseline, 0.6 mg/dL), and glucose 123 mg/dL. Alanine aminotransferase (ALT) was 20 U/L, aspartate aminotransferase (AST) 51 U/L, total bilirubin 3.1 mg/dL, direct bilirubin 0.4 mg/dL, albumin 3.4 g/dL, total protein 6.8 g/dL, and alkaline phosphatase 79 U/L.

White blood cell count was 10,000/μL, with 67% neutrophils, 18% lymphocytes, 7% monocytes, 0% eosinophils, and 0% basophils. Hemoglobin was 5.3 g/dL, hematocrit 15.3%, and platelets 2,000/μL. Repeat laboratory studies confirmed severe anemia and thrombocytopenia. Reticulocyte count was 9.06%. Prothrombin time was 15.1 seconds, activated partial thromboplastin time was 37.7 seconds, and international normalized ratio was 1.2. Fibrinogen was 445 mg/dL, and haptoglobin was < 8 mg/dL. Urinalysis showed 3+ blood, 2+ protein, negative leukocyte esterase, negative nitrites, 25 to 50 red bloods/high-powered field, 0 to 4 white blood cells/high-powered field, and 0 to 2 granular casts/high-powered field.

Question 1

In this case, what is the most appropriate next best test?

A. Ferritin measurement

B. Peripheral blood smear examination

C. Cyanocobalamin measurement

Question 2

Based on the patient’s clinical presentation and the peripheral blood smear film, what is the most likely diagnosis?

A. HELLP syndrome (hemolysis with a microangiopathic blood smear, elevated liver enzymes, low platelet count)

B. Disseminated intravascular coagulation

C. Thrombotic thrombocytopenic purpura or hemolytic uremic syndrome

Question 3

What test would aid in making the correct diagnosis prior to treatment?

A. Assess mutation in complement proteins

B. von Willebrand factor multimer analysis

C. ADAMTS13 analysis

Guest Editors

Syed A. Abutalib, MD, Assistant Director, Hematology & Bone Marrow Transplantation Service, Cancer Treatment Centers of America, Zion, Illinois

Jean M. Connors, MD, Assistant Professor, Harvard Medical School, Hematology Division, Brigham and Women’s Hospital/Dana-Farber Cancer Institute, Boston


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Microangiopathic Hemolytic Anemia and Thrombocytopenia Answers

Question 1: In this case, what is the most appropriate next best test?

Correct Answer: B. Peripheral blood smear examination.

Expert Perspective

In the appropriate clinical setting, information obtained from a carefully examined peripheral blood smear film is indispensable. The peripheral blood...


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