Laboratory work up of a 20 year old woman with newly diagnosed unprovoked PE demonstrates no thrombophilia, except for presence of heterozygosity for FVL, determined by PCR-based genetic testing.

Which of the following statements regarding heterozygous FVL is correct?

You are seeing an asymptomatic, healthy, 17 year old female who has never had a thrombotic event. She wants to be tested for FVL prior to starting OCPs because her 24 year old sister had a birth control associated DVT and was found to be heterozygous for FVL. The test of the 17 year old indicates that she does not have FVL.

Which of the following statements is correct?

Which of the following patients should be treated with indefinite anticoagulation therapy?

Which of the following patients has a clear indication for the placement of an IVC filter?

A 21 year old otherwise healthy woman presents to the ED wit a 1 day history of left leg swelling and pain. The patient was treated for an extensive left leg DVT when she was 14 after started OCPs. Her medical history is otherwise negative. The patient is not currently using any form of estrogen or progestin, does not smoke an has no first degree family history of VTE. She runs 3 miles most days of the week. Pregnancy test is negative.

Compression US reveals left sided iliofemoral DVT, and the patient is started on therapeutic doses of LMWH.

She has no chest pain or SOB and O2 sats are normal.

What additional testing is indicated in the management of this patient?

A full-term infant develops induration and erythema of the skin within a few hours after birth. Over the next 2 days, the lesions worsen and and become necrotic, and the infant is transferred to the NICU for close monitoring. The infant otherwise appears well and is active. A sepsis workup is negative. Blood samples are collected to evaluate for protein C or Protein S deficiency.

What is the most appropriate next step in the management of this patient?

A 35-day old, previously healthy boy is evaluated in the ED for a seizure. PE reveals minor bruising, but child abuse is not suspected. There is no evidence of meningitis, but erythrocytes are detected in the spinal fluid. A CT shows bilateral parenchymal hemorrhages. There is no family history of unusual bleeding. The child did not bleed at circumcision, but there was delayed detachment of the umbilical cord with subsequent bleeding of the umbilical stump that lasted for several days. PT, aPTT and thrombin time are all normal.

Which of the following is the most likely diagnosis?

A 15 yo male is evaluated in the hospital for excessive bleeding 2 weeks after undergoing nasal sinus surgery. The patient did not have excessive blood loss at surgery, but bleeding followed by hematemesis began 1 day after surgery. Tho days later, surgery was again performed, and the area was explored and wound beds cauterized; no abnormal bleeding vessels were detected. Hemoglobin at that time was 12; preoperative level was 16. Five days later he developed a serosanguinous drainage followed by frank bleeding. Hb was 7 and 2 U of PRBC were administered. Laboratory studies show a normal platelet count.

The patient has no family history of excessive bleeding and a vague personal history of bleeding after dental surgery 25 years ago. He has a strong family history of coronary artery disease.

Which of the following tests should be done at this time to evaluate this patient's condition?

A 1-day old male infant is evaluated for abnormal bleeding after circumcision. The infant is hemodynamically stable, and the acute bleeding has been controlled with cautery, local hemostatic agents, and dressing. The mother is 29 years old, and the child is her first. She had menorrhagia as an adolescent that resolved with OCPs; she has no other personal or family history of abnormal bleeding. The father has no personal or family history of abnormal bleeding. Evaluation of the child reveals a normal complete blood count. The PT is 2 sec above the normal range, and the aPTT is 85 sec; on mixing studies, both correct.

Which of the following is the most appropriate additional evaluation for this infant?

A 14-year-old African American male comes into your office complaining of sudden onset fatigue, jaundice, and dark urine. He has recently visited family in sub-Saharan Africa and started feeling tired two days into the trip. Since malaria is endemic in the area he was visiting, he took prophylactic anti-malarials during the trip. He denies fevers or recent illness.

Blood tests are taken and the results are as follows: liver enzymes are normal with the exception of elevated indirect bilirubin; Hgb: 10 g/dL; Hct: 32%; Reticulocyte count 5%; MCV: 95 fL. DAT is negative.

Which of the following is most correct concerning this disease?

You are asked to evaluate anemia in a 18 year old hospitalized female. She has a 5-year history of JIA and presented with fatigue and dyspnea. Her C-reactive protein and erythrocyte sedimentation (ESR) rate are markedly elevated. She denies blood in her urine, stool or heavy menstrual periods.

Her CBC shows:

Hemoglobin 9.2 g/dL (12-16 g/dL)

Hematocrit 28% (35-48%)

MCV 80 fL (80-100 fL)

WBC 9.9 K/μl (4.5-10.5 K/μL)

Platelet count 194 K/μL (150-450 K/μL)

Which of the following additional laboratory test results is consistent with the most likely etiology of her anemia?

An 11-year-old female was admitted during the night for the treatment of a sickle cell pain crisis. The members of the team seem to be frustrated that this is her third admission this month for a pain crisis, but she is started on a morphine PCA for management of her pain. She has no fever and her Hb on admission is 6.9 g/dl.

Which of the following is true concerning patients with sickle cell disease?

You are working in the local health department immunization and WIC clinic and a mother brings in a 6 month old boy for his immunizations. Mom reports that she wants him to have the immunizations but she does not want any formula because “everything upsets his stomach”; therefore, they have been feeding him milk from a neighbor’s goat, which the baby tolerates well. He appears thin, pale, and lethargic; is breathing rapidly; is tachycardic with a 2/6 systolic murmur; and has petechiae on his legs.

The automated CBC results are:

WBC = 2.0 x 103/ml with an ANC of 900

RBC = 1.5 x 106/ml

Hemoglobin = 4 g/dL

Hematocrit = 12%

MCV = 105 fL

Reticulocyte count = 0.5%

PLT count = 35,000 /ml

Which of the following is the likely etiology of his anemia?

A 19-year-old male patient is admitted to the ICU with chest pain, slurred speech, and left-sided weakness. Past medical history is significant for hypothyroidism and bipolar disorder. Home medications include valproic acid and levothyroxine. On physical exam, he is in mild distress, afebrile, and mildly hypertensive. Complete blood count shows a leukocyte count of 6,600/ μL, hemoglobin 6.5 g/ dL, and platelet count 16,000/ μL. Creatinine is 0.8 mg/ dL and LDH is 683 U/ L.

What is the most important initial step in the management of this patient?

An 8-year-old girl and her 9-year-old brother have multiple bleeding episodes and large hematomas following any minor trauma. Clinicians from the Bleeding Disorders Clinic have made a diagnosis of severe Type 3 von Willebrand disease. Which test is likely normal in these siblings?

A 4 year boy is scheduled for a T&A by ENT. A screening PT/PTT shows a prolonged aPTT of 42 sec and he is referred for pre-operative clearance. He has no history of bleeding or significant bruising and no family history of bleeding disorders. He was circumcised at birth with no bleeding complications.

Further evaluation in the bleeding disorders clinic is most likely going to discover which of the following laboratory abnormalities?

A male patient with factor VIII deficiency is an expectant father and wants to know whether his child could also have hemophilia A. Assume the mother of the unborn child and her family have no history of bleeding.

What is the likely chance the patient has a male baby with factor VIII deficiency?

During a routine pre-school physical you discover that a 4-year-old African-American girl has an ANC of 800. Her mother reports that she is in excellent health, she has never had a clinically significant infection, and she has no history of mouth sores. You perform serial blood counts twice per week for 6 weeks, and on each occasion her ANC is between 600 and 1000.

The most likely diagnosis for this child is:

An 8-month-old girl is brought to the emergency department by her parents because of yellowing of her skin. PE shows scleral icterus, diffuse jaundice, and splenomegaly. A peripheral blood smear is shown. The reticulocyte count is 4%. Her parents state that three relatives have a similar condition.

As a result of this disease process, the patient would be at greatest risk of developing which in her teenage years?

A 6-month-old girl is referred for a prolonged prothrombin time. She was evaluated before planned placement of bilateral myringotomy tubes for repeated ear infections. During the last 3 months, she has had four episodes of bilateral otitis media and has been treated successfully with antibiotics each time. Her parents deny any family history of bleeding, and the patient has not had any abnormal bruising or bleeding with immunizations. The patient is exclusively breastfed. She has never undergone surgery and is otherwise healthy. The findings of her examination are normal. Prothrombin time is measured at 20 sec (upper limit of normal 13.8 sec). Her partial thromboplastin time and complete blood count findings are normal.

Of the following, the MOST likely cause of her abnormal laboratory finding is: