7.00 Hematological Disorders Listings
Effective date 12/15/04
“Chronicity” - persistence of the condition for at least 3 months
Medical records must report laboratory findings on more than one examination over that 3-month period
“Medically acceptable imaging” includes:
- X-ray imaging
- Computerized axial tomography (CAT scan)
- Magnetic resonance imaging (MRI)
- Myelography – x-ray of spinal cord after injecting substance into the space just below the lining of the spinal cord – allows better visualization of structures
- Radionuclear bone scans – x-ray imaging of bone after injection of radioactive dye
“Appropriate” - the technique used is the proper one to support the evaluation and diagnosis of the impairment
7.01 Category of Impairments, Hematological Disorders
7.02. Chronic anemia (hematocrit persisting at 30 percent or less due to any cause).
Anemia
- Low red blood cell count resulting in a deficiency of the oxygen-carrying part of blood
- Measured by red blood cell count, hemoglobin, or hematocrit found in a complete blood cell count (CBC) – blood test
- Symptoms include weakness, fatigue, shortness of breath, palpitations (irregular and forceful beating of the heart), dizziness and feeling faint
- Signs of advanced anemia include pallor (paleness), rapid heart rate, and low blood pressure
- Gradual development of anemia (slow decrease in red blood cells), even to very low levels, may be well tolerated in individuals with healthy cardiovascular (heart and blood vessels) and pulmonary (lung) systems
- Prolonged anemia may cause heart failure because the heart has to work extra hard to compensate for the lower oxygen delivery to the body
With:
A. Requirement of one or more blood transfusions on an average of at least once every 2 months;
This “average” should be over a 12 month time period.
Or
B. Evaluation of the resulting impairment under criteria for the affected body system.
Any organ or part of the body may be affected, but frequently the heart, lungs, kidneys, spleen, or brain are damaged, which may be irreversible.
An example of an “affected body system” might be the cardiovascular system if long-standing uncorrected anemia causes chronic heart failure.
7.05 Sickle cell disease, or one of its variants.
Sickle cell disease
- A chronic hemolytic (red blood cells break apart) anemia associated with sickle-shaped red blood cells and other abnormal hemoglobins, such as in thalassemia.
- Abnormally shaped red blood cells have a tendency to hemolyze (break apart)
- Tests, such as hemoglobin electrophoresis (lab test that detects abnormal hemoglobin), should be in the medical record to confirm the diagnosis
- Aplastic episodes, also known as aplastic crisis - occurs when the bone marrow temporarily shuts down or the spleen captures red blood cells, causing a sudden fall in hemoglobin.
- “Major visceral episodes” include meningitis (inflammation of the lining of the brain), osteomyelitis (bone infection), pulmonary (lung) infections or infarctions (lack of blood flow causing tissue to break down), cerebrovascular accidents (lack of blood flow to the brain causing tissue to break down), congestive heart failure (poor heart function resulting in fluid overload in the body), and kidney damage.
Variants of sickle cell disease include those with one hemoglobin “S” gene and one other abnormal hemoglobin gene, such as hemoglobin “SC” disease or hemoglobin “S” beta thalassemia.
Classic sickle cell anemia is two hemoglobin “S” genes.
Sickle cell trait is one hemoglobin “S” gene and one normal gene (“AS”) – a “carrier” state, and usually does not cause symptoms.
With:
A. Documented painful (thrombotic) crises occurring at least three times during the 5 months prior to adjudication;
Vasoocclusive (thrombotic) episodes, also known as pain crisis - occurs when red blood cells stick in the small blood vessels causing breakdown of tissue due to lack of oxygen resulting in pain
A true pain crisis implies substantial pain that usually requires a visit to an emergency room, and intravenous or intramuscular administration of pain medication to relieve the pain.
Or
B. Requiring extended hospitalization (beyond emergency care) at least three times during the 12 months prior to adjudication;
“Extended” - at least a 24-hour hospital admission
These extended hospitalizations must be documented in the medical records at least 3 times in the 12 months prior to the date of the disability decision.
Or
C. Chronic, severe anemia with persistence of hematocrit of 26 percent or less;
“Chronic” and “persistence” - hematocrit levels must be 26 percent of less on repeated blood draws over a three month period
Keep in mind Social Security’s definition of disability – this "persistence" should also be expected to last at least 12 months before meeting this listing.
Or
D. Evaluate the resulting impairment under the criteria for the affected body system.
Any organ or part of the body may be affected with the heart, lungs, kidneys, spleen, bone, and brain most likely to be damaged; can be irreversible damage.
7.06 Chronic thrombocytopenia (due to any cause) with platelet counts repeatedly below 40,000/cubic millimeter.
Thrombocytopenia – low platelet count
Platelets, which are found in the blood, help blood to clot
Platelet counts are found in a complete blood count (CBC) lab test
Normal platelet counts are usually over 150,000/cubic millimeter
Listing requires platelet counts below 40,000/cubic millimeter on repeated blood tests
With:
A. At least one spontaneous hemorrhage, requiring transfusion, within 5 months prior to adjudication;
“Spontaneous hemorrhage” - bleeding not caused by trauma or injury
Spontaneous bleeding without trauma or injury does not usually occur until platelets counts drop below 20,000/cubic millimeter, and is common when platelet counts drop below 10,000/cubic millimeter.
The spontaneous hemorrhage must be treated with transfusion to meet this listing.
“Within 5 months prior to adjudication” - within the 5 months prior to the disability decision
Or
B. Intracranial bleeding within 12 months prior to adjudication.
Intracranial bleeding means bleeding inside the brain or between the brain and the skull.
Bleeding in either area is considered a hemorrhagic stroke.
Note that this listing does not require that the intracranial bleeding be spontaneous; therefore, this bleeding can be due to trauma or injury.
7.07 Hereditary telangiectasia with hemorrhage requiring transfusion at least three times during the 5 months prior to adjudication.
Hereditary telangiectasia - also known as Osler-Weber-Rendu syndrome
A rare inherited disorder where blood vessels form lacking a normal capillary connection between an artery and a vein
This abnormal connection is called a telangiectasia in small blood vessels and an arteriovenous malformation (AVM) in large blood vessels
These abnormal connections can easily rupture and cause bleeding into the skin, mucous membranes, and organs.
“During the 5 months prior to adjudication” - within the 5 months prior to the disability decision
7.08 Coagulation defects (hemophilia or a similar disorder) with spontaneous hemorrhage requiring transfusion at least three times during the 5 months prior to adjudication.
Coagulation defects - disorders of blood clotting, which can result in bleeding
May be treated with antihemophilic globulin (AHG) concentrate (clotting factors) and transfusion of fresh frozen plasma, which contains clotting factors
Prophylactic (preventive) therapy with AHG does not by itself reflect the severity of the condition
Hemophilia - inherited bleeding disorder caused by coagulation (clotting) factor deficiencies
Clotting factors - proteins produced by the body to help blood clot and stop bleeding
Blood coagulation disorders include:
- Factor V deficiency
- Factor VII deficiency
- Factor VIII deficiency – Hemophilia A
- Factor IX deficiency – Hemophilia B
- Factor X deficiency
- Factor XI deficiency
- Factor XII deficiency
- Factor XIII deficiency
- Afibrinogenemia
- Dysfibrinogenemia
- Protein C and S deficiencies
- Von Willebrand disease - a clotting protein called von Willebrand factor is deficient or defective; most common inherited bleeding disorder
“Spontaneous hemorrhage” - bleeding not caused by trauma or injury
“Transfusion” - refers to transfusion of packed red blood cells, and not fresh frozen plasma
“During the 5 months prior to adjudication” - within the 5 months prior to the disability decision
Must be documented by appropriate lab tests, such as:
- Partial thromboplastin time (PTT) – may be prolonged
- Prothrombin time (PT), which may be reported as INR prothrombin (International Normalized Ratio PT) - may be prolonged
- Bleeding time, clotting time, and thrombin time - may be prolonged
- Assay/analysis of specific coagulation (clotting) factors – may be decreased
7.09 Polycythemia vera (with erythrocytosis, splenomegaly, and leukocytosis or thrombocytosis). Evaluate the resulting impairment under the criteria for the affected body system.
Polycythemia vera – a myeloproliferative disorder - overproduction by the bone marrow of mainly red blood cells, but also white blood cells and platelets
Erythrocytosis – overproduction of red blood cells; majority have hematocrits over 55 percent
Splenomegaly – enlarged spleen
Leukocytosis – overproduction of white blood cells
Thrombocytosis – overproduction of platelets
Can increase blood viscosity resulting in sluggish blood flow and thrombotic events – blood clotting
Affected body systems and associated conditions include:
- Deep venous thrombosis (DVT) – blood clot in a deep vein
- Blood clot in arteries causing tissue damage and even gangrene
- Pulmonary embolus – blood clot in lung
- Kidney failure from clot in kidney vein or artery
- Intestinal ischemia from blood clot in mesenteric vein draining the intestines
- Heart attack due to clot in arteries feeding the heart
- Stroke due to clot in arteries feeding the brain
- Budd-Chiari syndrome – liver condition resulting from blood clot in vein draining the liver
- Hypertension (high blood pressure)
- Gout
7.10 Myelofibrosis (myeloproliferative syndrome).
Myelofibrosis - bone marrow, which produces red blood cells, white blood cells and platelets, is replaced by non-functioning fibrous tissue
Myeloproliferative syndrome - causes sustained abnormally increased formation of cells by the bone marrow
May be a primary condition or secondary due to such conditions as leukemia, lymphoma or progression of polycythemia vera.
Diagnosed by bone marrow biopsy
With:
A. Chronic anemia. Evaluate according to the criteria of 7.02;
Or
B. Documented recurrent systemic bacterial infections occurring at least 3 times during the 5 months prior to adjudication;
“Systemic” implies sepsis with life-threatening widespread infection in the blood or tissues requiring hospitalization
“At least 3 times during the 5 months prior to adjudication” implies 3 hospitalizations for infection during the 5 months before the disability decision is made
Or
C. Intractable bone pain with radiologic evidence of osteosclerosis.
“Intractable” - persistent pain not responding to treatment
“Radiologic evidence” – findings on x-ray or other imaging studies
“Osteosclerosis” – hardening of bone or bone marrow; shows up as area of whiteness on x-ray where the pathologic bone density has increased
7.15 Chronic granulocytopenia (due to any cause).
“Chronic Granulocytopenia” – abnormally low number of granular white blood cells persisting for at least 3 months on repeated blood tests
Produces increased risk of infection
Possible causes of granulocytopenia:
- Bone marrow disorders due to myelofibrosis, aplastic anemia, malignancy, and radiation
- Autoimmune diseases such as systemic lupus, scleroderma, and rheumatoid arthritis
- Felty syndrome – the triad of rheumatoid arthritis, splenomegaly and neutropenia
- Congenital syndromes from birth
With both A and B:
A. Absolute neutrophil counts repeatedly below 1,000 cells/cubic millimeter;
Neutrophils - white blood cells, which help prevent infection
The absolute neutrophil count is calculated by multiplying the total white blood cell count (WBC) by the percentage of neutrophils found in a complete blood count (CBC).
Normal neutrophil counts range between 3,000 and 6,000 cells/cubic millimeter of blood.
When the absolute neutrophil count falls below 1,000 cells/cubic millimeter of blood, there is a significant risk of increased bacterial infections.
And
B. Documented recurrent systemic bacterial infections occurring at least 3 times during the 5 months prior to adjudication.
“Systemic” implies sepsis with life-threatening widespread infection in the blood or tissues requiring hospitalization
“At least 3 times during the 5 months prior to adjudication” implies 3 hospitalizations for infection during the 5 months before the disability decision is made
7.17 Aplastic anemias with bone marrow or stem cell transplantation. Consider under a disability for 12 months following transplantation; thereafter, evaluate according to the primary characteristics of the residual impairment.
Aplastic anemia – a form of anemia in which the bone marrow fails to produce an adequate number of blood cells, including red blood cells, white blood cells, and platelets
Diagnosed by bone marrow biopsy
May be caused by an autoimmune disorder, radiation, chemicals, drugs, and malignancy
Severe cases are treated with bone marrow or stem cell transplantation.
Stem cell transplantation usually refers to transplantation of stem cells (precursors of blood cells) collected from the peripheral blood.
When stem cells are collected from the bone marrow prior to transplantation, this refers to bone marrow transplantation.
Individuals are considered disabled for 12 months from the date of the transplantation; though, often times, Social Security will grant an earlier onset of disability if the medical records show the applicant was too sick to work before the transplantation occurred.