1/30/18 Resident Morning Report – Shortness of Breath, Hemoptysis, Fever, Anemia, Bilateral Infiltrates, Hypoxemia, and Diffuse Alveolar Hemorrhage

CC: Shortness of Breath, Hemoptysis


HPI: This is a 23 year-old male with no past medical history who presents with progressive fatigue, pleuritic chest pain, shortness of breath, and hemoptysis for 2 months.  Initially his cough was productive with occasional blood-streaked sputum but progressed to frank hemoptysis in the last 3-4 weeks. Additionally, he reports subjective intermittent fevers and chills for 3-4 weeks.  Also notable is a 30 lbs unintentional weight loss over 2 months.  Exam shows patient saturating at 78% on room air, tachypneic to 30, febrile to 38.6, and tachycardic to 128.  Patient has diffuse crackles and wheezes from bases to mid lung fields but no rashes or joint findings.  His labs were notable for a normocytic anemia and leukocytosis with left shift.  CXR showed bilateral diffuse disease (see below) with CT demonstrating ground-glass opacities in a central distribution.   On BAL,  diffuse blood and secretions throughout all segments of lobes on both side of the lungs were visualized.  A diagnosis of Diffuse Alveolar Hemorrhage was made with the underlying etiology being Systemic Lupus Erythematosus based off other findings of nephritic syndrome with renal biopsy demonstrating Lupus Nephritis Class 3, +ANA, +dsDNA, low complements.


Morning Report Pearls:

Clinical suspicion for Diffuse Alveolar Hemorrhage should always be present when diffuse infiltrates on Chest X-ray are seen in the setting of anemia, respiratory failure and hemoptysis.  Of note, one third of patients will not have hemoptysis!


Diagnosis is made on BAL when it demonstrates serial samples that are progressively hemorrhagic.   Yield is higher if performed within the first 48 hours of symptoms.


There are many causes of DAH with overlapping pathophysiology.  DAH can result from small vessel inflammation resulting in alveoli necrosis followed by leakage of RBCs into the lung, bleeding into the alveolar space as a result of diffuse alveolar damage (ARDS pathologies), or bland hemorrhage resulting from etiologies like coagulopathies.


Pulmonary Capillaritis Conditions:

  • Systemic Vasculitis (GPA, MPA, Cryoglobunemia, HSP, IgA vasculitis)
  • Connective Tissue Disorders (SLE, MCTD, RA, APLS)
  • Drugs like Retinoic acid or PTU
  • Lung transplant rejection


Bland Pulmonary Hemorrhage Conditions:

  • Left sided heart failure
  • Mitral stenosis or mitral regurgitation
  • Anticoagulants or coagulopathies like DIC
  • Pulmonary Veno-occlusive Disease
  • Endocarditis
  • Drugs like Marcobid


Diffuse Alveolar Damage Conditions:

  • Anything that can cause ARDS like infections
  • Bone marrow transplantation
  • Crack cocaine inhalation
  • SLE


When having a patient with DAH, always consider the Pulmonary-Renal Syndromes which is defined as DAH with Glomerulonephritis.  This is one of many reasons to always obtain a urinalysis on these patients.  The pulmonary-renal syndromes really narrow the above list to Vasculitis and Connective Tissue Disorders if present.

1/16/18 Resident Morning Report – Arm Swelling, Bruising, and Pain Secondary to Coagulopathy Caused by Acquired Factor VIII Inhibitor

CC: Left Arm Swelling and Pain

HPI: 84 y/o Caucasian male with history of CAD s/p CABG, HTN, HLD, CKD stage 2, and BPH presented with a 2 day history of swelling and excruciating pain in the L arm following a routine lab draw from a vein in the L hand. Swelling had begun at the site and expanded to include the entirety of his arm. Overlying bruising emerged with increasing pain. He also noted bruising of the internal thighs incidentally but denied trauma at either site. He had not history of bleeding diathesis diagnosed in him or a family member and had no reported easy bruising or bleeding previously. He had no recent medication changes. Physical exam was notable for a swollen and tender L arm with erythema and extensive ecchymoses extending from the wrist to the elbow. Range of motion was intact as were peripheral pulses, even in the affected arm. Notably, the patient had bruising on the extremities and on his trunk and abdomen. Physical exam was not concerning for compartment syndrome of the arm given the aforementioned findings, though the team closely monitored for signs of impending arterial compression. Initial laboratory evaluation revealed an elevated partial thromboplastin time (PTT) but a normal prothrombin time (PT) and international normalized ratio (INR). Mixing studies were performed, and the PTT did not correct suggesting the presence of an inhibitor. Factor VIII levels were checked and were significantly lower than the lower limit of normal. Factor VIII inhibitor level was detected at 35 Bethesda Units. This confirmed the presence of an acquired factor VIII inhibitor.

Morning Report Pearls:

Bleeding disorders can be divided primarily into broad categories, those that affect primary hemostasis and those that affect secondary hemostasis. Disorders of both primary and secondary hemostasis can be either acquired or inherited.

Some Causes of Primary Hemostatic Disorders, Acquired

  • Thrombocytopenia (many causes including ITP, TTP, etc.)
  • Cirrhosis
  • DIC or Sepsis
  • Malignancy, especially leukemia or lymphoma
  • Myeloproliferative or Myelodysplastic Disorders
  • Uremia
  • Medications/Drugs, including NSAIDs, aspirin, etc

Some Causes of Primary Hemostatic Disorders, Inherited

  • von Willebrand Disease
  • Congenital Afibrinogenemia
  • Genetic Platelet Disease, including Wiskott-Aldrich Syndrome, Bernard-Soulier Disease, Glanzmann Thrombasthenia

Some Causes of Secondary Hemostatic Disorders, Acquired

  • Vitamin K Deficiency
  • Medications, including Warfarin and Heparin
  • Liver Failure or Cirrhosis
  • DIC or Sepsis
  • Fat Malabsorption
  • Acquired Factor Deficiencies, including Acquired Factor VIII Deficiency
  • Malignancy, especially leukemia or lymphoma

Some Causes of Secondary Hemostatic Disorders, Inherited

  • Hemophilias (A, B, or C)
  • Cystic Fibrosis Resulting in Malabsorption

When evaluating a patient with a suspected bleeding disorder, physical exam can be very informative in narrowing the diagnosis. Patients with primary hemostatic disorders (platelets) tend to present with mucosal or cutaneous bleeding; epistaxis or gingival bleeding; petechiae, especially in dependent areas; immediate bleeding post-injury; and menorrhagia/metomenorrhoagia. Patients with secondary hemostatic disorders (coagulation factors) tend to present with large palpable ecchymoses; hemorrhage into joints or muscles; delayed bleeding given preservation of platelet function and number; and post-operative bleeding.

Secondary hemostatic disorders affect PT, INR, and PTT depending on the factors involved. Whenever PT or PTT are affected due to concern for bleeding diathesis, a mixing study is appropriate. In this case, PTT was prolonged. An example of a PTT mixing study is below:

The results of this mixing study led to concern for an acquired Factor VIII inhibitor, which was confirmed. Of note, lupus anticoagulant was ruled out on lab testing.

Causes of Acquired Factor VIII Inhibitor

  • Malignancy
  • Pregnancy
  • Autoimmune Disease (including SLE)
  • Clonal Lymphoproliferative Disorders

12/5/17 Resident Morning Report – Flank pain, Shortness of breath, Kidney Injury, Bilateral Hydronephrosis, Pleural Effusion, Retroperitoneal Fibrosis, Adenocarcinoma

CC: flank pain

HPI: 56 year old Korean man with no past medical history reports 2 months of worsening bilateral flank pain. He states it was of sudden onset with gradual worsening of the pain over 2 months.  It radiates to upper back, but there is no radiation to groin.  Intensity is 10/10 at its worst, intermittent, sharp in quality, and worse when lying down. In addition, he complains of subjective fevers, abdominal swelling, shortness of breath and decreased exercise tolerance. He is only able to walk up 1 flight of stairs when he previously could walk multiple flights. He denies chest pain, palpitations, lower extremity swelling, and orthopnea. He also denies any changes to urination, dysuria, frequency, gross hematuria or difficulty urinating. The patient has been seen at several outside emergency departments, however, he has not had further work up due to lack of funding. He reports he has always been healthy and has never been hospitalized, never had surgeries, and never been treated for any diseases. Exam is pertinent for decrease breath sounds at the bilateral lung bases with dullness to percussion.  The patient also has moderate tenderness to palpation to bilateral flanks.



BUN/Cr: 19/1.61

K: 5.3

Pro-BNP: 277

UA: moderate blood, 4-5 RBCs

Urine Cr 45

Urine urea 219

Feurea 41%


IgG 1046 mg/dL (690-1600)

  • IgG-1 = 690
  • IgG-2 = 181
  • IgG-3 = 15
  • IgG-4 = 7.5


Abdominal US:

Moderate bilateral hydronephrosis. No obstructing lesion is identified within the renal collecting systems.

Bilateral pleural effusions.




Pleural Fluid:

Yellow color

Cell count = 1073 (PMN 5%, lymph 23%, 9% mono, 63% other)

Protein 4.3, ratio: 4.3/6.0 = 0.72

LDH 221, ratio: 221/154 = 1.44

Glucose 72

Gram stain negative, no organisms

Fungal culture negative


Pleural Fluid Cytology:


CT Abdomen/Pelvis with Contrast:


The patient had evidence of bilateral hydronephrosis and pleural effusions.  CT A/P demonstrated retroperitoneal fibrosis encasing the bilateral renal arteries and abdominal aorta, with hepatosplenomegaly, and mild T12 vertebral body compression fracture. Urology performed cystocospy with ureteral stent placement however renal function worsened requring IR to place nephrostomy tubes.  Pleural fluid was obtained with cytology positive for adenocarcinoma.  The patient’s IgG4 levels were not elevated.  Overall, it was though that the patient had retroperitoneal fibrosis secondary to malignancy which was discovered to be signet ring cell adenocarcinoma of the gastrointestinal tract.


Morning Report Pearls:

Retroperitoneal fibrosis is a rare entity that involves fibrotic tissue encasing abdominal organs including the ureters. Hydronephrosis without any obvious causative lesion on abdominal ultrasound is a classic finding that was seen in this patient.


It can be primary or secondary in nature.  Primary disease includes IgG4 disease. Secondary causes include: Drugs, Biologics, Malignancy, Infections, Autoimmune disease, Radiation, Retroperitoneal hemorrhage, and Surgery