<clinical_study>
  <!-- This xml conforms to an XML Schema at:
    https://clinicaltrials.gov/ct2/html/images/info/public.xsd -->
  <required_header>
    <download_date>ClinicalTrials.gov processed this data on September 20, 2023</download_date>
    <link_text>Link to the current ClinicalTrials.gov record.</link_text>
    <url>https://clinicaltrials.gov/ct2/show/NCT01672918</url>
  </required_header>
  <id_info>
    <org_study_id>120184</org_study_id>
    <secondary_id>12-I-0184</secondary_id>
    <nct_id>NCT01672918</nct_id>
  </id_info>
  <brief_title>Fluorodeoxyglucose Imaging Studies to Detect Lymphoma</brief_title>
  <official_title>Use of Fluorodeoxyglucose Positron Emission Tomography With Computed Tomography for the Evaluation of Autoimmune Lymphoproliferative Syndrome Lymphadenopathy Suggestive of Lymphoma</official_title>
  <sponsors>
    <lead_sponsor>
      <agency>National Institute of Allergy and Infectious Diseases (NIAID)</agency>
      <agency_class>NIH</agency_class>
    </lead_sponsor>
  </sponsors>
  <source>National Institutes of Health Clinical Center (CC)</source>
  <brief_summary>
    <textblock>
      Background:&#xD;
&#xD;
      - Autoimmune lymphoproliferative syndrome (ALPS) is a genetic disorder of the lymph system.&#xD;
      People with ALPS often have swollen lymph nodes, especially in the neck and armpit. They also&#xD;
      have a much higher risk of developing lymphoma. It is not always easy to determine whether&#xD;
      the swollen lymph nodes are caused by ALPS or by lymphoma. Researchers want to see whether&#xD;
      different imaging studies can show the difference between ALPS and lymphoma. The studies used&#xD;
      will be positron emission tomography (PET) and computed tomography (CT). Researchers will use&#xD;
      a drug called fluorodeoxyglucose (FDG) to look at the lymph nodes.&#xD;
&#xD;
      Objectives:&#xD;
&#xD;
      - To see how well imaging studies can distinguish between swollen lymph nodes caused by ALPS&#xD;
      or by lymphoma.&#xD;
&#xD;
      Eligibility:&#xD;
&#xD;
        -  Individuals must be 5 years of age or older and enrolled on the National Institutes of&#xD;
           Health natural history study of ALPS.&#xD;
&#xD;
        -  Participants should either have lymphoma or have symptoms that suggest possible&#xD;
           lymphoma.&#xD;
&#xD;
      Design:&#xD;
&#xD;
        -  Participants will be screened with a physical exam and medical history. Blood and urine&#xD;
           samples will be collected.&#xD;
&#xD;
        -  Participants will have an FDG-PET/CT scan. It will be performed according to standard&#xD;
           procedures.&#xD;
&#xD;
        -  If the results of the scan do not show lymphoma, participants will stay on the study for&#xD;
           1 year for clinical follow up. They may have a second FDG-PET/CT scan if there is a&#xD;
           change in symptoms. Such changes include further enlargement of lymph nodes, unexplained&#xD;
           fevers, or weight loss.&#xD;
&#xD;
        -  If the results of the scan show evidence of new or worsening lymphoma, treatment on this&#xD;
           study will end. Further tests based on clinical symptoms, including a lymph node biopsy,&#xD;
           may be done under the ALPS natural history study to rule out or make a diagnosis of&#xD;
           lymphoma.&#xD;
    </textblock>
  </brief_summary>
  <detailed_description>
    <textblock>
      The Autoimmune Lymphoproliferative Syndrome (ALPS) is an inherited disorder associated with&#xD;
      defective lymphocyte apoptosis, which is clinically characterized by prominent nonmalignant&#xD;
      lymphadenopathy, hepatosplenomegaly, and overt autoimmune diseases such as hemolytic anemia,&#xD;
      autoimmune thrombocytopenia, and neutropenia. Additionally, ALPS patients have a&#xD;
      significantly increased risk of developing non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma&#xD;
      (HL). The diagnosis of lymphoma is particularly troublesome in ALPS because many ALPS&#xD;
      manifestations overlap with clinical features suggestive of lymphoma. Therefore, individuals&#xD;
      with ALPS may undergo repeated biopsies during the course of the disease. Fluorodeoxyglucose&#xD;
      positron emission tomography combined with computed tomography (FDG-PET/CT) is a noninvasive&#xD;
      test that may help us discriminate benign from malignant lymphadenopathy in patients with&#xD;
      ALPS.&#xD;
&#xD;
      For patients with ALPS and clinical symptoms suggestive of lymphoma, such as a sudden&#xD;
      increase in focal lymphadenopathy and/or systemic B symptoms associated with lymphoma, we&#xD;
      want to investigate whether FDG-PET/CT is useful in determining a plan of action by assisting&#xD;
      in locating the most active lymph node and determining whether a surgical biopsy is&#xD;
      warranted. Under this protocol, FDG-PET/CT scans will be done to rule out lymphoma. A lymph&#xD;
      node biopsy may be necessary to determine the pathology of the lymph node and will not be&#xD;
      done for research purposes alone.&#xD;
    </textblock>
  </detailed_description>
  <overall_status>Withdrawn</overall_status>
  <start_date>August 6, 2012</start_date>
  <completion_date>February 27, 2015</completion_date>
  <study_type>Observational</study_type>
  <has_expanded_access>No</has_expanded_access>
  <study_design_info>
    <time_perspective>Prospective</time_perspective>
  </study_design_info>
  <enrollment type="Actual">0</enrollment>
  <condition>Autoimmune Lymphoproliferative Syndrome</condition>
  <condition>Lymphoma</condition>
  <eligibility>
    <criteria>
      <textblock>
        -  INCLUSION CRITERIA:&#xD;
&#xD;
        To qualify for enrollment, patients must meet all of the following:&#xD;
&#xD;
          1. Fulfill current criteria for the diagnosis of ALPS, which includes documented chronic&#xD;
             nonmalignant lymphadenopathy and/or splenomegaly, and either greater than or equal to&#xD;
             1.5% T-cell receptor alpha/beta+ DNTs in the peripheral blood or confirmed RAS&#xD;
             mutation with or without elevated alpha/beta DNTs.&#xD;
&#xD;
          2. Be enrolled in ALPS natural history protocol #93-I-0063.&#xD;
&#xD;
          3. Have 1 or more of the following:&#xD;
&#xD;
               1. Sudden enlargement of at least 1 lymph node or group of lymph nodes over&#xD;
                  baseline.&#xD;
&#xD;
               2. Systemic symptoms suspicious for lymphoma (i.e., loss of weight, loss of&#xD;
                  appetite, fatigue, night sweats, fever, and pruritus).&#xD;
&#xD;
               3. A histologically proven diagnosis of lymphoma or other malignancy.&#xD;
&#xD;
          4. Be 5 years of age or older.&#xD;
&#xD;
        EXCLUSION CRITERIA:&#xD;
&#xD;
        Patients will be excluded if any of the following is present:&#xD;
&#xD;
          1. Concurrent proven infection or inflammatory disease (e.g., sarcoidosis), which itself&#xD;
             often shows increased FDG uptake by PET and which could interfere with the&#xD;
             interpretation of study results.&#xD;
&#xD;
          2. Hyperglycemia (regardless of etiology) determined by fasting glucose of &gt;200 mg/dL&#xD;
&#xD;
          3. Weight in excess of 400 lb, which will exceed the weight limit for the scanner table.&#xD;
&#xD;
          4. Pregnancy or breast-feeding. For women of childbearing potential, a negative urine or&#xD;
             serum pregnancy test is required within 24 hours prior to an FDG-PET/CT scan.&#xD;
      </textblock>
    </criteria>
    <gender>All</gender>
    <minimum_age>5 Years</minimum_age>
    <maximum_age>99 Years</maximum_age>
    <healthy_volunteers>No</healthy_volunteers>
  </eligibility>
  <overall_official>
    <last_name>V. Koneti Rao, M.D.</last_name>
    <role>Principal Investigator</role>
    <affiliation>National Institute of Allergy and Infectious Diseases (NIAID)</affiliation>
  </overall_official>
  <removed_countries>
    <country>United States</country>
  </removed_countries>
  <reference>
    <citation>Sneller MC, Wang J, Dale JK, Strober W, Middelton LA, Choi Y, Fleisher TA, Lim MS, Jaffe ES, Puck JM, Lenardo MJ, Straus SE. Clincal, immunologic, and genetic features of an autoimmune lymphoproliferative syndrome associated with abnormal lymphocyte apoptosis. Blood. 1997 Feb 15;89(4):1341-8.</citation>
    <PMID>9028957</PMID>
  </reference>
  <reference>
    <citation>Straus SE, Sneller M, Lenardo MJ, Puck JM, Strober W. An inherited disorder of lymphocyte apoptosis: the autoimmune lymphoproliferative syndrome. Ann Intern Med. 1999 Apr 6;130(7):591-601. doi: 10.7326/0003-4819-130-7-199904060-00020.</citation>
    <PMID>10189330</PMID>
  </reference>
  <reference>
    <citation>Avila NA, Dwyer AJ, Dale JK, Lopatin UA, Sneller MC, Jaffe ES, Puck JM, Straus SE. Autoimmune lymphoproliferative syndrome: a syndrome associated with inherited genetic defects that impair lymphocytic apoptosis--CT and US features. Radiology. 1999 Jul;212(1):257-63. doi: 10.1148/radiology.212.1.r99jl40257.</citation>
    <PMID>10405750</PMID>
  </reference>
  <verification_date>February 27, 2015</verification_date>
  <study_first_submitted>August 23, 2012</study_first_submitted>
  <study_first_submitted_qc>August 23, 2012</study_first_submitted_qc>
  <study_first_posted type="Estimate">August 27, 2012</study_first_posted>
  <last_update_submitted>December 4, 2019</last_update_submitted>
  <last_update_submitted_qc>December 4, 2019</last_update_submitted_qc>
  <last_update_posted type="Actual">December 5, 2019</last_update_posted>
  <responsible_party>
    <responsible_party_type>Sponsor</responsible_party_type>
  </responsible_party>
  <keyword>PET/CT</keyword>
  <keyword>Lymphoma</keyword>
  <keyword>Lymphadenopathy</keyword>
  <keyword>ALPS</keyword>
  <condition_browse>
    <!-- CAUTION:  The following MeSH terms are assigned with an imperfect algorithm            -->
    <mesh_term>Lymphoma</mesh_term>
    <mesh_term>Lymphadenopathy</mesh_term>
    <mesh_term>Autoimmune Lymphoproliferative Syndrome</mesh_term>
    <mesh_term>Syndrome</mesh_term>
  </condition_browse>
  <!-- Results have not yet been posted for this study                                          -->
</clinical_study>


download date: ClinicalTrials.gov processed this data on September 20, 2023
link text: Link to the current ClinicalTrials.gov record.
url: https://clinicaltrials.gov/ct2/show/NCT01672918
org study id: 120184
secondary id: 12-I-0184
nct id: NCT01672918
lead sponsor: 
Background:

      - Autoimmune lymphoproliferative syndrome (ALPS) is a genetic disorder of the lymph system.
      People with ALPS often have swollen lymph nodes, especially in the neck and armpit. They also
      have a much higher risk of developing lymphoma. It is not always easy to determine whether
      the swollen lymph nodes are caused by ALPS or by lymphoma. Researchers want to see whether
      different imaging studies can show the difference between ALPS and lymphoma. The studies used
      will be positron emission tomography (PET) and computed tomography (CT). Researchers will use
      a drug called fluorodeoxyglucose (FDG) to look at the lymph nodes.

      Objectives:

      - To see how well imaging studies can distinguish between swollen lymph nodes caused by ALPS
      or by lymphoma.

      Eligibility:

        -  Individuals must be 5 years of age or older and enrolled on the National Institutes of
           Health natural history study of ALPS.

        -  Participants should either have lymphoma or have symptoms that suggest possible
           lymphoma.

      Design:

        -  Participants will be screened with a physical exam and medical history. Blood and urine
           samples will be collected.

        -  Participants will have an FDG-PET/CT scan. It will be performed according to standard
           procedures.

        -  If the results of the scan do not show lymphoma, participants will stay on the study for
           1 year for clinical follow up. They may have a second FDG-PET/CT scan if there is a
           change in symptoms. Such changes include further enlargement of lymph nodes, unexplained
           fevers, or weight loss.

        -  If the results of the scan show evidence of new or worsening lymphoma, treatment on this
           study will end. Further tests based on clinical symptoms, including a lymph node biopsy,
           may be done under the ALPS natural history study to rule out or make a diagnosis of
           lymphoma.
The Autoimmune Lymphoproliferative Syndrome (ALPS) is an inherited disorder associated with
      defective lymphocyte apoptosis, which is clinically characterized by prominent nonmalignant
      lymphadenopathy, hepatosplenomegaly, and overt autoimmune diseases such as hemolytic anemia,
      autoimmune thrombocytopenia, and neutropenia. Additionally, ALPS patients have a
      significantly increased risk of developing non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma
      (HL). The diagnosis of lymphoma is particularly troublesome in ALPS because many ALPS
      manifestations overlap with clinical features suggestive of lymphoma. Therefore, individuals
      with ALPS may undergo repeated biopsies during the course of the disease. Fluorodeoxyglucose
      positron emission tomography combined with computed tomography (FDG-PET/CT) is a noninvasive
      test that may help us discriminate benign from malignant lymphadenopathy in patients with
      ALPS.

      For patients with ALPS and clinical symptoms suggestive of lymphoma, such as a sudden
      increase in focal lymphadenopathy and/or systemic B symptoms associated with lymphoma, we
      want to investigate whether FDG-PET/CT is useful in determining a plan of action by assisting
      in locating the most active lymph node and determining whether a surgical biopsy is
      warranted. Under this protocol, FDG-PET/CT scans will be done to rule out lymphoma. A lymph
      node biopsy may be necessary to determine the pathology of the lymph node and will not be
      done for research purposes alone.
time perspective: Prospective
criteria: 
gender: All
minimum age: 5 Years
maximum age: 99 Years
healthy volunteers: No
last name: V. Koneti Rao, M.D.
role: Principal Investigator
affiliation: National Institute of Allergy and Infectious Diseases (NIAID)
country: United States
citation: Sneller MC, Wang J, Dale JK, Strober W, Middelton LA, Choi Y, Fleisher TA, Lim MS, Jaffe ES, Puck JM, Lenardo MJ, Straus SE. Clincal, immunologic, and genetic features of an autoimmune lymphoproliferative syndrome associated with abnormal lymphocyte apoptosis. Blood. 1997 Feb 15;89(4):1341-8.
PMID: 9028957
citation: Straus SE, Sneller M, Lenardo MJ, Puck JM, Strober W. An inherited disorder of lymphocyte apoptosis: the autoimmune lymphoproliferative syndrome. Ann Intern Med. 1999 Apr 6;130(7):591-601. doi: 10.7326/0003-4819-130-7-199904060-00020.
PMID: 10189330
citation: Avila NA, Dwyer AJ, Dale JK, Lopatin UA, Sneller MC, Jaffe ES, Puck JM, Straus SE. Autoimmune lymphoproliferative syndrome: a syndrome associated with inherited genetic defects that impair lymphocytic apoptosis--CT and US features. Radiology. 1999 Jul;212(1):257-63. doi: 10.1148/radiology.212.1.r99jl40257.
PMID: 10405750
responsible party type: Sponsor
mesh term: Lymphoma
mesh term: Lymphadenopathy
mesh term: Autoimmune Lymphoproliferative Syndrome
mesh term: Syndrome