--- title: "Part V, Subpart iii, Chapter 9" document: "M-21-1" section: "Part V, Subpart iii, Chapter 9" canonical: "https://veteranai.co/va-regulations/m21-1/v.iii.9" source: "https://www.knowva.ebenefits.va.gov/system/templates/selfservice/va_ssnew/help/customer/locale/en-US/portal/554400000001018/content/554400000180513/M21-1-Part-V-Subpart-iii-Chapter-9-Hematologic-and-Lymphatic-Systems" updated: "2026-09-07T14:37:17.922998Z" --- June 14, 2019 ### V.iii.9.1.a. Definition: Sickle Cell Disease ** _Sickle cell disease_** is a generic term for all disorders characterized by the presence of sickle hemoglobin in the red blood cells and includes * sickle cell anemia * sickle cell trait, and * other hemoglobinopathies such as * sickle cell thalassemia, and * sickle-hemoglobin C disease. **Notes:** * The phenomenon of sickling of red blood cells is a hereditary abnormality that of itself usually produces few ill effects. * Sickle cell trait alone is generally not a ratable disability, as provided in [38 CFR 4.117, diagnostic code (DC) 7714](). ### V.iii.9.1.b. Definition: Sickle Cell Disease **_Sickle cell anemia_** is a hereditary and familial disorder characterized clinically by symptoms of * anemia * arthritis * leg ulcers, and * acute attacks of pain. **Note:** The age of onset is generally early childhood. ### V.iii.9.1.c. Inheritance of Sickle Cell Trait Inheritance of sickle cell trait may be from one or both parents.If sickle hemoglobin is inherited from one parent and normal hemoglobin from the other, the combination is referred to as sickle cell trait. **Note:** Except for unusual circumstances, this is a benign asymptomatic condition and is not associated with increased morbidity. ### V.iii.9.1.d. Inheritance of Sickle Cell Anemia The inheritance of sickle hemoglobin from each parent results in sickle cell anemia.Sickle cell anemia is usually accompanied by * moderate to severe anemia, and * appropriate clinical signs and symptoms, such as * enlargement of the heart * abnormalities of the musculoskeletal system * bone and joint pain, and/or * fever. ### V.iii.9.1.e. Characteristics of Sickle Cell Anemia Sickle cell anemia is a morbid state characterized by hemolytic anemia and the following manifestations * the presence of peculiar sickle-shaped, or oat-shaped, red blood cells * signs of excessive blood destruction and active blood formation, and * repeated vaso-occlusive episodes. ### V.iii.9.1.f. Mechanism of Inheritance of Sickle Hemoglobin The presence of sickle hemoglobin, a variant of the normal hemoglobin in human red blood cells, is subject to the usual mechanisms of biologic inheritance. 2. Other Hematologic and Lymphatic Conditions This topic contains information about hemic and lymphatic conditions, including * assigning a permanent and total (P&T) evaluation for amyloid light chains (AL) amyloidosis (primary amyloidosis) * review examinations of non-Hodgkin’s Lymphoma (NHL) and other persistent cancers * pyramiding of NHL and chronic lymphocytic leukemia (CLL) * evaluating monoclonal gammopathy of undetermined significance (MGUS) * definitions of bone marrow and stem cell transplant * rating schedule update, and * historical P&T evaluations. September 15, 2025 ### V.iii.9.2.a. Assigning a P&T Evaluation for AL Amyloidosis Assign a permanent and total (P&T) evaluation for amyloid light chains (AL) amyloidosis (primary amyloidosis). AL amyloidosis is considered incurable and progressive.**Notes:** * Evaluate AL amyloidosis under [38 CFR 4.117, DC 7717](). * Consider ancillary benefits associated with the award of P&T disability evaluations. * AL amyloidosis is a disability that is presumptively associated with herbicide exposure. **References:** For more information on * rating disabilities associated with herbicide exposure, see [M21-1, Part VIII, Subpart i, 1.C](), and * addressing claims for P&T evaluation based on malignancy prognosis, see [M21-1, Part V, Subpart ii, 3.D.4.i](). ### V.iii.9.2.b. Review Examinations of NHL and Other Persistent Cancers When evaluating the need for a review examination at the two-year period prescribed in the rating schedule under [38 CFR 4.117, DC 7715](), following discontinuance of the treatment phase for non-Hodgkin’s lymphoma (NHL) or any other persistent cancer with a high mortality rate, consider that the various therapeutic treatment modalities may continue at intervals greater than the review period indicated in the rating schedule. If the disease has actively persisted for several years, thoroughly examine the medical record to determine whether the disease is * actually in remission, or * still active and being regularly treated over extended periods of time. Do **_not_** schedule a review examination unless the record clearly shows a long-term and stable remission. **Important:** Consider assigning a P&T evaluation when a provision under [38 CFR 3.327(b)(2)]() applies or as otherwise warranted under the provisions of [M21-1, Part V, Subpart ii, 3.D.4](). **Reference:** For more information on when not to schedule a review examination, see [M21-1, Part IV, Subpart ii, 1.A.1.d](). ### V.iii.9.2.c. Pyramiding of CLL and NHL Do not assign separate evaluations for chronic lymphocytic leukemia (CLL) ([38 CFR 4.117, DC 7703]()) and NHL ([38 CFR 4.117, DC 7715]()). They are cancers of the same body system and assignment of multiple evaluations would be pyramiding. In cases where both cancers are diagnosed, assign the appropriate evaluation using a hyphenated DC. **References:** For more information on * pyramiding, see [M21-1, Part V, Subpart ii, 3.D.2.b](), and * presumptive service connection (SC) for CLL and NHL, see [M21-1, Part VIII, Subpart i, 1.C.1](). ### V.iii.9.2.d. Evaluating MGUS ** _Monoclonal gammopathy of undetermined significance_** (MGUS) is an asymptomatic, pre-malignant disorder characterized by monoclonal plasma cell proliferation in the bone marrow and absence of end organ damage.Evaluate MGUS under [38 CFR 4.117, DC 7712]().**Notes:** * MGUS is generally asymptomatic, but it can sometimes present with tingling or numbness in the hands or feet. * Routine blood tests can lead to MGUS detection. **Important:** MGUS does not have to be compensable to qualify for presumptive SC due to herbicide exposure.**Reference:** For more information on presumptive SC for MGUS based on herbicide exposure, see * [M21-1, Part VIII, Subpart i, 1.A](), and * [M21-1, Part VIII, Subpart i, 1.C](). ### V.iii.9.2.e. Definition: Bone Marrow and Stem Cell Transplant A **_bone marrow transplant_** , also called a **_stem cell transplant_** , is a procedure used to infuse healthy cells, called stem cells, into the body to replace damaged or diseased bone marrow. The process of infusing healthy cells into the body to replace diseased tissue may be referred to in a variety of manners based on the donor source and the type of tissue used. The following procedures all satisfy the criteria for the 100-percent rating under [38 CFR 4.117](): * stem cell transplant * bone marrow transplant * bone marrow stem cell transplant * peripheral blood transplant, and * peripheral blood stem cell transplant. ### V.iii.9.2.f. Rating Schedule Update The criteria for rating disabilities of the hematologic and lymphatic systems in [38 CFR 4.117]() were most recently updated effective * December 9, 2018, and * October 23, 1995. The purpose of these updates was to * incorporate medical advances * update medical terminology * add disabilities not previously included, and * refine rating criteria. **Note:** These updates were not liberalizing changes in the rating criteria. **Reference:** For more information on these and other historical changes to [38 CFR 4.117](), see * [38 CFR 4, Appendix A]() * [38 CFR 4.117 (Historical)](), and * the [Board of Veterans Appeal Research Tools]() website for historical [38 CFR 4.117](). ### V.iii.9.2.g. Historical P&T Evaluations Historical policy guidance in existence prior to the December 9, 2018, rating schedule update directed the assignment of P&T evaluations for CLL and multiple myeloma. P&T evaluations assigned under the prior version of the rating schedule with application of those policies are protected under [38 CFR 3.951(a)](). P&T evaluations were directed * from November 6, 2003, to December 9, 2018, for CLL, with diagnosis being the sole requirement unless the condition was considered cured via treatment with a bone marrow transplant, and * from January 28, 2003, to December 9, 2018, for multiple myeloma unless an exceptional situation was present in which inactive multiple myeloma was documented. **Important:** * In all situations, the evidence of record must be analyzed to determine the treatment plan and prognosis for active malignancy associated with CLL or multiple myeloma. As directed in [M21-1, Part V, Subpart ii, 3.D.4](), assign a P&T evaluation for active malignancy when warranted, whether the disability is evaluated under historical or current rating criteria. * Revisions to [38 CFR 4.117, DCs 7703 and 7712]() effective on December 9, 2018, provide criteria for scheduling review examinations for CLL and multiple myeloma unless there is clear evidence establishing a terminal prognosis.