Question 51: Did the recipient experience any types of bleeding (since the date of last report)?

Due to WAS-associated thrombocytopenia, bleeding episodes are frequently observed. Indicate whether the recipient experienced any types of bleeding since the date of last report. If “yes” continue with question 52. If “no” continue with question 73.

Question 52: Is epistaxis present?

Indicate if epistaxis, bleeding from the nose, is present. If “yes” continue with question 53. If “no” continue with question 54.

Question 53: Is epistaxis prominent?

Indicate if epistaxis is prominent. A prominent feature is generally well documented, closely followed, and treated. Epistaxis may be recurrent, life-threatening, or require a transfusion. Continue with question 54.

Question 54: Is upper GI hemorrhage present?

Hemorrhage in the upper gastrointestinal tract includes bleeding that occurs above the duodenojejunal juncture of the small intestines. Signs of upper GI hemorrhage include vomiting blood (hematemesis) and black “tarry” stool (melena). As with other sources of blood loss, symptoms can include light headedness and fainting, weight loss, jaundice, and pain. Indicate if upper GI hemorrhage is present. If “yes” continue with question 55. If “no” continue with question 56.

Question 55: Is upper GI hemorrhage prominent?

Indicate if upper GI hemorrhage is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 56.

Question 56: Is lower GI hemorrhage/rectal bleeding present?

Hemorrhage in the lower gastrointestinal tract includes bleeding that occurs below the duodenojejunal juncture of the small intestines. Signs of lower GI hemorrhage include bright red blood in stool (hematochezia) and spontaneous rectal bleeding without defecation. Lower GI hemorrhage presents with symptoms similar to those associated with upper GI hemorrhage. Depending on the location and severity of hemorrhage, upper and lower GI bleeds may have similar presentations. Indicate if there is lower GI hemorrhage/rectal bleeding present. If “yes” continue with question 57. If “no” continue with question 58.

Question 57: Is lower GI hemorrhage/rectal bleeding prominent?

Indicate if lower GI hemorrhage/rectal bleeding is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 58.

Question 58: Is hemarthrosis present?

Hemarthrosis refers to bleeding into joint spaces leading to swelling and pain. Suspected hemarthrosis is often confirmed with a joint aspiration. Indicate if hemarthrosis is present. If “yes” continue with question 59. If “no” continue with question 60.

Question 59: Is hemarthrosis prominent?

Indicate if bleeding into joint spaces is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 60.

Question 60: Is hematuria present?

Hematuria refers to blood in the urine and can be further specified as gross hematuria or microscopic hematuria. Gross hematuria is visibly noticeable while microscopic hematuria is only apparent when examined with a microscope. Hematuria may either be asymptomatic or be associated with symptoms such as painful urination and changes in urination frequency. Indicate if hematuria is present. If “yes” continue with question 61. If “no” continue with question 62.

Question 61: Is hematuria prominent?

Indicate if blood in the urine is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 62.

Question 62: Is intracranial hemorrhage present?

Intracranial hemorrhage involves bleeding within the cranium and may present with symptoms including nausea and vomiting, headache, and altered consciousness. CT scans and other imaging modalities are commonly used to visualize intracranial bleeding. Indicate if intracranial hemorrhage is present. If “yes” continue with question 63. If “no” continue with question 64.

Question 63: Is intracranial hemorrhage prominent?

Indicate if intracranial hemorrhage is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 64.

Question 64: Is oral bleeding present?

Indicate if oral bleeding is present. If “yes” continue with question 65. If “no” continue with question 66.

Question 65: Is oral bleeding prominent?

Indicate if oral bleeding is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 66.

Question 66: Is subcutaneous bleeding present?

Indicate if there is bleeding under the skin, commonly identified by bruising (petechiae, purpura, ecchymosis). If “yes” continue with question 67. If “no” continue with question 68.

Question 67: Is subcutaneous bleeding prominent?

Indicate if bleeding under the skin is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 68.

Question 68: Is subdural hematoma present?

The dura mater is the outermost membrane that encloses the brain and spinal cord, keeping in the cerebrospinal fluid. A subdural hematoma is a collection of blood below the dura mater. Subdural hematoma symptoms include: headache, decreased consciousness, and motor deficits. CT scans are commonly used to visualize subdural bleeding. Indicate if a subdural hematoma is present. If “yes” continue with question 69. If “no” continue with question 70.

Question 69: Is subdural hematoma prominent?

Indicate if subdural hematoma is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 70.

Question 70: Is other bleeding present?

Indicate if bleeding other than listed in questions 52-69 is present. If “yes” continue with questions 71 and 72. If “no” continue with question 73.

Question 71: Is other bleeding prominent?

Indicate if other bleeding is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 72.

Question 72: Specify other bleeding?

Specify the type of other bleeding indicated in question 70. Continue with question 73.

Question 73: Did the recipient experience any of the following autoimmune / inflammatory disorders (since the date of the last report)?

Due to aberrant lymphocyte function associated with WAS, autoimmune and inflammatory disorders are common. Indicate “yes” if the recipient experienced autoimmune / inflammatory disorder(s) and continue with questions 74-105 to further specify the disorder(s). If “no” continue with question 106.

Question 74: Is arthralgia present?

Indicate if the recipient experienced joint pain. If “yes” continue with question 75. If “no” continue with question 76.

Question 75: Is arthralgia prominent?

Indicate if arthralgia is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 76.

Question 76: Is chronic arthritis present?

Chronic arthritis refers to persistent joint inflammation, leading to pain, swelling, and stiffness often with reduced movement. Indicate if chronic arthritis is present. If “yes” continue with question 77. If “no” continue with question 78.

Question 77: Is chronic arthritis prominent?

Indicate if chronic arthritis is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 78.

Question 78: Is autoimmune hemolytic anemia present?

Autoimmune hemolytic anemia refers to the destruction (hemolysis) of red blood cells by the recipient’s own immune system. Anemia results when the recipient’s marrow is unable to sufficiently produce replacement red blood cells. Laboratory studies are the most common method of disease detection, usually involving a complete blood cell count and peripheral blood smear. Indicate if autoimmune hemolytic anemia is present. If “yes” continue with question 79. If “no” continue with question 80.

Question 79: Is autoimmune hemolytic anemia prominent?

Indicate if autoimmune hemolytic anemia is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 80.

Question 80: Is idiopathic thrombocytopenic purpura (ITP) present?

ITP refers to decreased platelet counts with normal bone marrow and the absence of other thrombocytopenia causes. Indicate if ITP is present. If “yes” continue with question 81, if “no” continue with question 82.

Question 81: Is idiopathic thrombocytopenic purpura (ITP) prominent?

Indicate if ITP is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 82.

Question 82: Is inflammatory bowel disease present?

Inflammatory bowel disease (IBD) is a general term referring to inflammation anywhere along the lining of the gastrointestinal tract. Ulcerative colitis and Crohn’s disease are the two major types of IBD and commonly manifest with abdominal cramping and abnormal bowel movements including constipation, diarrhea, and the passage of mucus and/or blood. Complete blood counts, stool studies, and serologic studies can be performed to better characterize symptoms and exclude other disorders on the differential diagnosis. Imaging studies, especially endoscopies are used to diagnosis and monitor IBD. Indicate if inflammatory bowel disease is present. If “yes” continue with question 83. If “no” continue with question 84.

Question 83: Is inflammatory bowel disease prominent?

Indicate if inflammatory bowel disease is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 84.

Question 84: Is juvenile rheumatoid arthritis present?

Juvenile rheumatoid arthritis is a condition of autoimmune joint inflammation causing joint pain, swelling, and stiffness, with childhood onset. Indicate if juvenile rheumatoid arthritis is present. If “yes” continue with question 85. If “no” continue with question 86.

Question 85: Is juvenile rheumatoid arthritis prominent?

Indicate if juvenile rheumatoid arthritis is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 86.

Question 86: Is nephritis present?

Nephritis refers to inflammation of the kidneys and may be further specified based on the area of kidney involvement and whether the inflammation is acute or chronic. Tests performed to assess and monitor kidney function include BUN and creatinine. In certain cases, a renal biopsy may be performed. Indicate if nephritis is present. If “yes” continue with question 87, if “no” continue with question 88.

Question 87: Is nephritis prominent?

Indicate if nephritis is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 88.

Question 88: Is neutropenia present?

Neutropenia refers to a decreased number of neutrophils in the blood (ANC < 1.0 × 109/L). The risk of infection increases as the neutrophil count decreases. Indicate if the recipient is neutropenic. If “yes” continue with question 89, if “no” continue with question 90.

Question 89: Is neutropenia prominent?

Indicate if neutropenia is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 90.

Question 90: Is sclerosing cholangitis present?

Sclerosing cholangitis refers to inflammation and subsequent scarring and destruction of the bile ducts, eventually leading to liver damage. Imaging studies of the bile duct (cholangiography), as well as liver function tests such as aminotransferase and alkaline phosphatase are used to diagnose and monitor the disease. Indicate if sclerosing cholangitis is present. If “yes” continue with question 91, if “no” continue with question 92.

Question 91: Is sclerosing cholangitis prominent?

Indicate if sclerosing cholangitis is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 92.

Question 92: Is cerebral vasculitis present?

Vasculitis refers to inflammation of the vasculature, including both veins and arteries. Vasculitis may impact blood vessels of any size, from capillaries and arterioles to the great truncal vessels. It is typically caused by autoimmunity.

Cerebral vasculitis refers to inflammation involving vasculature of the brain. Indicate if the recipient had cerebral vasculitis. If “yes” continue with question 93, if “no” continue with question 94.

Question 93: Is cerebral vasculitis prominent?

Indicate if cerebral vasculitis is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 94.

Question 94: Is coronary vasculitis present?

Coronary vasculitis refers to inflammation involving vasculature of the heart. Indicate if coronary vasculitis is present. If “yes” continue with question 95, if “no” continue with question 96.

Question 95: Is coronary vasculitis prominent?

Indicate if coronary vasculitis is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 96.

Question 96: Is renal vasculitis present?

Renal vasculitis refers to inflammation involving vasculature of the kidney. Indicate if renal vasculitis is present. If “yes” continue with question 97, if “no” continue with question 98.

Question 97: Is renal vasculitis prominent?

Indicate if renal vasculitis is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 98.

Question 98: Is skin vasculitis present?

Indicate if the recipient has inflammation involving vasculature of the skin. If “yes” continue with question 99, if “no” continue with question 100.

Question 99: Is skin vasculitis prominent?

Indicate if skin vasculitis is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 100.

Question 100: Is other vasculitis present?

Indicate if other types of vasculitis are present than those listed in questions 92-99. If “yes” continue with question 101, if “no” continue with question 103.

Question 101: Is other vasculitis prominent?

Indicate if other vasculitis is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 102.

Question 102: Specify other vasculitis

Specify the other vasculitis indicated in question 100. Continue with question 103.

Question 103: Is any other disorder present?

Indicate if the recipient had an autoimmune / inflammatory disorder not listed in questions 74-105. If “yes” continue with question 104, if “no” continue with question 106.

Question 104: Is any other disorder prominent?

Indicate if the other disorder from question 103 is prominent. A prominent feature is generally well documented, closely followed, and treated. Continue with question 105.

Question 105: Specify other disorder

Specify the other disorder indicated in question 103. Continue with question 106.

Last modified: Sep 11, 2015

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