Hematologic System



Hematologic System





6-A. Anemia: Hypoproliferative (Low Reticulocyte Count)


Nutrient Deficiency

Iron deficiency



  • Chronic blood loss


  • Pregnancy


  • Dietary deficiency


  • Malabsorption



    • Subtotal gastrectomy


    • Malabsorption syndromes


  • Hemoglobinuria or hemosiderinuria



    • Intravascular hemolysis


  • Hemodialysis

Vitamin B12 deficiency



  • Dietary deficiency (rare)


  • Impaired absorption



    • Insufficient intrinsic factor



      • Pernicious anemia


      • Gastrectomy (total or partial)


      • Gastric mucosal injury (e.g., lye ingestion)


      • Congenital


  • Malabsorption syndromes



    • Sprue, tropical or nontropical


    • Ileal resection



    • Regional ileitis


    • Infiltrative intestinal disease (e.g., lymphoma)


    • Chronic pancreatitis


    • Familial selective malabsorption


    • Drug-induced malabsorption


  • Competitive absorption



    • Diphyllobothrium latum (fish tapeworm)


    • Blind-loop syndromes


  • Increased requirements



    • Pregnancy


    • Neoplasia

Folate deficiency



  • Dietary deficiency (especially in alcoholics, infants)


  • Impaired absorption



    • Malabsorption syndromes



      • Sprue, tropical or nontropical


      • Whipple disease


      • Small intestinal resection


      • Infiltrative intestinal disease (e.g., lymphoma)


      • Scleroderma


      • Amyloidosis


    • Drug-induced malabsorption (anticonvulsants)


  • Increased requirements



    • Pregnancy


    • Infancy


    • Hemolytic anemia


    • Chronic exfoliative dermatitis


    • Neoplasia


    • Uremia


  • Impaired metabolism



    • Trimethoprim


    • Methotrexate


    • Pyrimethamine


    • Alcohol

Other nutritional deficiencies



  • Vitamin A


  • Pyridoxine (rare)


  • Vitamin C


  • Starvation


  • Protein deficiency (kwashiorkor)



Anemia of Chronic Disease

Chronic infection



  • Subacute bacterial endocarditis


  • Osteomyelitis


  • Chronic pyelonephritis


  • Chronic pulmonary infection (e.g., bronchiectasis)


  • Tuberculosis


  • Chronic fungal infection


  • Pelvic inflammatory disease

Chronic inflammatory diseases



  • Rheumatoid arthritis


  • Rheumatic fever


  • Systemic lupus erythematosus


  • Vasculitis


  • Inflammatory bowel disease


  • Acquired immunodeficiency syndrome (AIDS) or human immunodeficiency virus (HIV) infection

Malignancy

Chronic renal disease

Chronic liver disease

Chronic lung disease

Endocrine dysfunction



  • Hypothyroidism


  • Hyperthyroidism


  • Hypogonadism


  • Adrenal insufficiency


  • Panhypopituitarism


  • Hyperparathyroidism

Pregnancy


Bone Marrow Disorders

Congenital



  • Red cell aplasia (Diamond-Blackfan anemia)


  • Congenital dyserythropoietic anemias


  • Hereditary sideroblastic anemias

Aplastic



  • Pancytopenia (see 6-L)


  • Pure red cell aplasia



    • Congenital (Diamond-Blackfan anemia)


    • Acquired



      • Associated with thymoma, chronic lymphocytic leukemia, and so forth


      • Drug induced


Toxic



  • Megaloblastic



    • Antimetabolites (e.g., 5-fluorouracil, 6-thioguanine, 6-mercaptopurine, azathioprine)


  • Sideroblastic (e.g., alcohol, lead, isoniazid, chloramphenicol)


  • Aplastic (see 6-L)

Infiltrative, with or without fibrosis



  • Infection



    • Tuberculosis


    • Fungal disease


  • Gaucher and other lipid storage diseases


  • Malignancy



    • Hematologic (leukemia, lymphoma, myeloma)


    • Marrow metastases

Neoplastic



  • Leukemia, acute and chronic


  • Lymphoproliferative disorders



    • Lymphoma, with marrow involvement



      • Hodgkin disease


      • Non-Hodgkin lymphoma (NHL)


    • Plasma cell myeloma


    • Hairy cell leukemia


  • Myeloproliferative disorders



    • Agnogenic myeloid metaplasia with fibrosis


    • Essential thrombocythemia


    • Chronic myelogenous leukemia


  • Myelodysplastic syndromes



    • Refractory anemia (RA)


    • RA with ringed sideroblasts


    • RA with excess of blasts (RAEB)


    • RAEB in transformation


    • Chronic myelomonocytic leukemia


Other

Marathon runner’s anemia (physiologic)



References

1. Aboulafia DM, Mitsuyasu RT. Hematologic abnormalities in AIDS. Hematol Oncol Clin N Am. 1991;5:195-214.

2. Caro J. Anemia of chronic renal failure, p. 449. See Bibliography, 2.

3. Gregg, XT, Prchal, JT. Anemia of endocrine disorders, p. 454. See Bibliography, 2.


4. Means RT Jr. Anemias secondary to chronic disease and systemic disorders, p. 1445. See Bibliography, 2.

5. Beutler E. Iron deficiency, p. 551. See Bibliography, 2.

6. Herbert V. Hematologic complications of alcoholism I, II. Semin Hematol. 1980;17:164-176.

7. Dressendorfer RH, Wade CE, Amsterdam EA. Development of pseudoanemia in marathon runners during a 20-day road race. JAMA. 1981;246:1215-1218.

8. See Bibliography, 3.

9. Beutler E. Anemia resulting from other nutritional deficiencies, p. 555. See Bibliography, 2.


6-B. Anemia: Hyperproliferative (Increased Reticulocyte Count)


Blood Loss



Hypersplenism



Hemolytic Anemia

Hereditary



  • Membrane abnormalities



    • Hereditary spherocytosis


    • Hereditary elliptocytosis


    • Hereditary stomatocytosis


    • Acanthocytosis (abetalipoproteinemia)


    • Others


  • Enzyme deficiencies



    • Glucose-6-phosphate dehydrogenase


    • Pyruvate kinase


    • Others


  • Hemoglobinopathies



    • Qualitative



      • Sickle cell anemia


      • Hemoglobin C disease


      • Unstable hemoglobin disease


      • Others


    • Unbalanced chain synthesis



      • Thalassemias

Acquired



  • Nonimmune



    • Traumatic



      • Prosthetic valves and other cardiac abnormalities


      • March hemoglobinuria


      • Burns


      • Ionizing irradiation



    • Microangiopathic hemolytic anemia



      • Disseminated intravascular coagulation (DIC)


      • Thrombotic thrombocytopenic purpura (TTP)


      • Hemolytic uremic syndrome


      • Antiphospholipid antibody syndrome


      • Vasculitis


      • Malignant hypertension


    • Infectious agents



      • Malaria


      • Clostridium perfringens


      • Bartonella


      • Babesiosis


      • Others


    • Chemical agents



      • Naphthalene


      • Arsine


      • Copper


      • Chlorates


      • Venoms


      • Distilled water (intravenous)


      • Others


    • Paroxysmal nocturnal hemoglobinuria


    • Hypophosphatemia


    • Spur cell anemia (liver disease)


  • Immune



    • Warm antibody mediated



      • Incompatible blood transfusion


      • Hemolytic disease of newborn


      • Idiopathic


      • Secondary



        • Infectious



          • Viral [e.g., Epstein-Barr virus, cytomegalovirus (CMV)]


        • Collagen-vascular disorders



          • Systemic lupus erythematosus


          • Rheumatoid arthritis


        • Malignancy



          • Lymphoproliferative disorders


          • Solid tumors


        • Other



          • Sarcoidosis


          • Inflammatory bowel disease



      • Drugs (many)


  • Cold antibody mediated



    • Idiopathic


    • Secondary



      • Infectious


      • Viral (e.g., Epstein-Barr virus)


      • Mycoplasma pneumoniae


    • Malignancy


  • Paroxysmal cold hemoglobinuria



References

1. Baker KR, Moake J. Hemolytic anemia resulting from physical injury, p. 709. See Bibliography, 2.

2. Beutler E. Disorders of red cells resulting from enzyme abnormalities, infection with microorganisms, pp. 603, 723. See Bibliography, 2.

3. Packman CH. Hemolytic anemia resulting from immune injury, p. 729. See Bibliography, 2.


6-C. Polycythemia

Spurious



  • Decreased plasma volume (e.g., dehydration, burns)


  • “Stress” erythrocytosis (Gaisböck’s syndrome)

Secondary



  • Appropriate (associated with tissue hypoxia)



    • Decreased arterial partial pressure of oxygen (PO2)



      • Altitude


      • Chronic pulmonary disease


      • Alveolar hypoventilation


      • Cyanotic congenital heart disease


    • Normal arterial PO2



      • Carboxyhemoglobinemia (e.g., cigarette smoking)


      • Hemoglobinopathies (with an increased affinity for oxygen)


      • Cobalt ingestion


      • Red cell enzyme deficiencies


  • Inappropriate



    • Renal disorders



      • Hydronephrosis


      • Renal cysts


      • Post-renal transplant erythrocytosis


      • Renal cell carcinoma



    • Endocrine disorders



      • Cushing syndrome


      • Primary hyperaldosteronism


      • Pheochromocytoma


      • Androgen therapy


    • Hepatoma


    • Cerebellar hemangioblastoma


    • Uterine leiomyoma


    • Familial erythrocytosis (e.g., Chuvash polycythemia)


    • Ovarian dermoid cyst

Primary



  • Polycythemia rubra vera


  • Primary familial and congenital polycythemia



References

1. Means RT Jr. See Bibliography, 1.

2. Prchal JT, Beutler E. Primary and secondary polycythemias (erythrocytosis), p. 779. See Bibliography, 2.


6-D. Granulocytopenia

Infections



  • Viral



    • Influenza


    • Infectious mononucleosis


    • Infectious hepatitis


    • Rubella


    • Chickenpox


    • Smallpox


    • Poliomyelitis


    • Others


  • Bacterial



    • Overwhelming bacteremia


    • Typhoid fever


    • Tularemia


    • Brucellosis


  • Mycobacterial



    • Miliary tuberculosis


  • Rickettsial


  • Protozoan



    • Malaria

Chemical agents, drugs, physical agents



  • Predictable



    • Antineoplastic agents



    • Benzene


    • Ionizing radiation


  • Idiosyncratic



    • Aminopyrine


    • Anticonvulsants


    • Antibiotics (e.g., chloramphenicol)


    • Antithyroid drugs


    • Clozapine


    • Ethanol


    • Phenothiazines


    • Sulfonamides


    • Others (immune suppression of production, e.g., ibuprofen)

Systemic illness



  • Systemic lupus erythematosus


  • Felty syndrome


  • AIDS or HIV infection

Hypersplenism

Nutritional



  • Vitamin B12 or folate deficiency


  • Cachexia


  • Copper deficiency


  • Alcoholism

Bone marrow dysfunction or accelerated destruction



  • Acute leukemias (aleukemic)


  • Lymphoproliferative disorders


  • Myelofibrosis



    • Primary: myeloproliferative disorders


    • Secondary



      • Tumor infiltration


      • Infection


  • Aplastic anemia


  • Myelodysplastic syndromes

Immune neutropenia



  • Drug induced


  • Collagen vascular disease



    • Systemic lupus erythematosus


    • Rheumatoid arthritis


    • Sjögren syndrome


  • Neoplasia


  • Autoimmune neutropenia



    • Idiopathic


    • Drugs


    • Collagen vascular disease


Other



  • Neutropenia associated with splenomegaly (e.g., sarcoidosis)


  • Benign neutropenia of blacks


  • Chronic idiopathic neutropenia


  • Congenital neutropenia (e.g., Kostmann syndrome)


  • Cyclic neutropenia


  • Benign familial neutropenia



References

1. Dale DC. Neutropenia and neutrophilia, p. 907. See Bibliography, 2.

2. Budman DR, Steinberg AD. Hematologic aspects of systemic lupus erythematosus. Ann Intern Med. 1977;86:220-229.

3. Aboulafia DM, Mitsuyasu RT. Hematologic abnormalities in AIDS. Hematol Oncol Clin N Am. 1991;5:195-214.


6-E. Granulocytosis

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Jun 19, 2016 | Posted by in GENERAL & FAMILY MEDICINE | Comments Off on Hematologic System

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