Additional Hematologic Conditions

OT Dude Academy’s Free NBCOT® Exam Prep Course Hematology Additional Hematologic Conditions
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1 Disseminated Intravascular Coagulation (DIC)
2 Thrombocytopenia
3 Hereditary Hemochromatosis
4 Leukemia

Disseminated Intravascular Coagulation (DIC)

  • A systemic thrombohemorrhagic [increased blood clotting and severe bleeding] disorder that can be life-threatening.
  • Widespread activation of coagulation results in the intravascular formation of fibrin and thrombotic occlusion of small and midsize blood vessels, resulting in compromised blood supply to the organs, leading to organ failure.
  • The pathogenic pathway for DIC is the systemic formation of fibrin from increased generation of thrombin, simultaneous suppression of anticoagulation mechanisms, and the delayed removal of fibrin due to impaired fibrinolysis.1 
  • Conditions associated with DIC: sepsis, trauma, cancer, obstetrical complications, vascular disorders, reactions to toxins, and immunologic disorders.1 
  • COVID-19: “The coagulopathy associated with COVID-19 mimics other systemic coagulopathies that are regularly seen in severe infections, most notably disseminated intravascular coagulation (DIC). However, COVID-19 has specific clinical and laboratory features that are distinctly different from the ‘classical’ presentation of DIC.”2 3 4 
  • The primary symptom of DIC is bleeding.5 6 7 
  • Additonal symptoms: petechiae, purpura, cyanosis, fever, hypotension, acidosis, proteinuria, hypoxia, and wound bleeding (surgical or traumatic). Large subcutaneous hematomas and deep tissue bleeding may be seen. The average patient with DIC usually bleeds from at least three unrelated sites.8 9 10 11 
  • Medical Management: treating the underlying disease, blood transfusion, anticoagulants12 13 
  • Prognosis: poor – intensity and duration depend on the degree to the coagulation system is activated and the reversal of the underlying condition leading to DIC.1 
  • Occupational Therapy: Literature on DIC and OT is lacking. From a professional reasoning standpoint, active-DIC or acute-DIC is a life-threatening condition and patients will likely be in the ICU requiring intensive medical care. When patients become stable, e.g., after a blood transfusion, OT may be more appropriate for these patients with orders to prevent physical deconditioning. These patients may be good candidates for early mobilization, but more research is needed in this area.14 

Thrombocytopenia

Source15 16 

  • A life-threatening condition characterized by a low platelet count due to decreased platelet production increased platelet consumption or sequestration.
  • Common etiologies for decreased platelet production include bone marrow failure, bone marrow suppression, chronic alcohol abuse, congenital syndromes, infections, myelodysplastic syndrome, neoplastic marrow infiltration, and nutritional deficiencies.
  • Common etiologies for increased platelet consumption include autoimmune syndromes, DIC and severe sepsis, drug-induced thrombocytopenia, heparin-induced thrombocytopenia, immune thrombocytopenic purpura, infection, mechanical destruction, preeclampsia and vaccinations.
  • Sequestration and other causes include chronic alcohol abuse, dilutional thrombocytopenia, gestational thrombocytopenia, hypersplenism, liver disease, pseudothrombocytopenia, pulmonary emboli, and pulmonary hypertension.
  • There is no specific diagnostic test for many thrombocytopenias.
  • Can be acute or chronic as determined by history, physical examination, and lab studies.
  • Patients may have spontaneous bruising and purpura [skin discoloration and purple spots]
  • A low platelet count may cause spontaneous bleeding and constitute a hematologic emergency. Bleeding may be mucosal, intracranial, gastrointestinal, or genitourinary.
  • Signs and symptoms
    • Pregnant women: visual symptoms, headaches, abdominal pain, flu-like symptoms to rule out preeclampsia
  • Treatment: based on etiology, may treat secondary causes to normalize platelet counts. Examples include discontinuing drugs, treating the infection, immunoglobulin G replacement, and chemotherapy. Platelet transfusion can be used in cases of severe bleeding and unknown thrombocytopenia etiology. However, if the underlying cause is immune, platelet transfusion may not be helpful.
  • Occupational Therapy: Exercise may increase the risk of cerebral, intramuscular, and joint hemorrhage in thrombocytopenic patients.17 18 
    • One study provided OT to children undergoing hematopoietic stem cell transplant with thrombocytopenia to address their ADLs, fine motor skills, visual perception, cognition, and psychosocial well-being. Treatment approaches included neuromotor, sensory, development of milestones, biomechanical, cognitive-psychosocial, and visual-perceptual. There was no correlation between intensity of the intervention and platelet count. There were no major bleeding complications related to therapy interventions. There were 5 episodes of minor bleeding out of 346 total sessions.19 
    • In determining the appropriateness of therapy for patients with thrombocytopenia, factors that affect bleeding include platelet count, platelet function, comorbid medical problems, infection, fever, coagulopathy, and medications.
    • Patients with acutely developing medical problems should be put on temporary therapy hold.

Hereditary Hemochromatosis

Source20 

  • An autosomal recessive disorder that disrupts iron regulation resulting in excessive iron absorption from food in the body.
  • Men have a 24-fold increased rate compared to women.
  • Most often found in Caucasians.
  • Iron is essential for cell metabolism.
  • With hereditary hemochromatosis, there is a toxic accumulation of iron in vital organs.
    • Iron-overload cardiomyopathy causes cardiomyopathy, diastolic dysfunction, heart failure, dysrhythmias, and conduction defects.
    • Iron-overload hepatopathy results in liver cirrhosis.
  • Associated with malignancies, particularly hepatocellular carcinoma.
  • Symptoms: weakness, lethargy, joint inflammation, and impotence.
  • Complications include osteoporosis, cirrhosis, hepatocellular cancer, cardiomyopathy, dysrhythmias, diabetes mellitus, and hypogonadism.
  • Treatment: phlebotomy to reduce total body iron levels and achieve normal ferritin levels. Second-line treatment is iron chelation therapy for patients who are intolerant of phlebotomy.
  • Dietary modification is generally unnecessary. Iron supplements and vitamin C should be avoided. No data has shown dietary manipulation improves patient outcomes. Raw shellfish should be avoided because of Vibrio vulnificus, a bacteria that can cause fatal infection with high iron levels.21 
  • Occupational Therapy: Intervention may include the use of orthotics, and adaptive equipment, and addressing flexibility, strength, positioning, and fall prevention.22  Additional rehabilitation may include addressing transfers, functional mobility, dressing, range of motion, joint stiffness and pain, balance, dyspnea, fatigue, sleep, sexual activity, and functional goals.23 
    • Patients often have secondary conditions which may also benefit from OT such as arthritis, cardiomyopathy, diabetes, osteopenia, and osteoporosis.
    • Practice infection prevention when working with postoperative patients or patients with wounds.
    • Consider holding OT if phlebotomy results in critically low hemoglobin and hematocrit levels.

Leukemia

See Oncology topic.

  1. Levi, M., & Ten Cate, H. (1999). Disseminated intravascular coagulation. New England Journal of Medicine, 341(8), 586-592.[↩][↩][↩]
  2. Gando S, Levi M, Toh CH (2016) Disseminated intravascular coagulation. Nature Rev Dis Prim 2:16037[↩]
  3. Levi M (2020) COVID-19 coagulopathy vs disseminated intravascular coagulation. Blood Adv 4:2850[↩]
  4. Levi M, Thachil J (2020) Coronavirus disease 2019 coagulopathy: disseminated intravascular coagulation and thrombotic microangiopathy-either, neither, or both. Semin Thromb Hemost 46:781–784[↩]
  5. Marder VJ, Feinstein DI, Francis CW, Colman RW. Consumptive thrombohemorrhagic disorders. In: Colman RW, Hirsh J, Marder VJ, Salzman EW, eds. Hemostasis and thrombosis: basic principles and clinical practice. 3rd ed. Philadelphia: J.B. Lippincott, 1994:1023-63.[↩]
  6. Bone RC. Modulators of coagulation: a critical appraisal of their role in sepsis. Arch Intern Med 1992;152:1381-1389[↩]
  7. Müller-Berghaus G, ten Cate H, Levi MM. Disseminated intravascular coagulation. In: Verstraete M, Fuster V, Topol EJ, eds. Cardiovascular thrombosis: thrombocardiology and thromboneurology. 2nd ed. Philadelphia: Lippincott-Raven, 1998:781-801.[↩]
  8. Baker WF. Clinical aspects of disseminated intravascular coagulation: a clinician’s point of view. Semin Thromb Hemost 1989;15:1 – 57[↩]
  9. Bick RL, Baker WF. Disseminated intravascular coagulation. Hematol Pathol 1992;6:1 – 24.[↩]
  10. Bick RL. Disseminated intravascular coagulation: objective clinical and laboratory diagnosis, treatment and assessment of therapeutic response. Semin Thromb Hemost 1996;22:69 – 88.[↩]
  11. Bick RL. Disseminated intravascular coagulation and related syndromes: a clinical review. Semin Thromb Hemost 1988;14:299 – 338.[↩]
  12. Levi, M., Toh, C. H., Thachil, J., & Watson, H. G. (2009). Guidelines for the diagnosis and management of disseminated intravascular coagulation. British journal of hematology, 145(1), 24-33.[↩]
  13. Wada, H., Asakura, H., Okamoto, K., Iba, T., Uchiyama, T., Kawasugi, K., … & Japanese Society of Thrombosis Hemostasis/DIC subcommittee. (2010). Expert consensus for the treatment of disseminated intravascular coagulation in Japan. Thrombosis research, 125(1), 6-11.[↩]
  14. Zang, K., Chen, B., Wang, M., Chen, D., Hui, L., Guo, S., … & Shang, F. (2020). The effect of early mobilization in critically ill patients: A meta‐analysis. Nursing in critical care, 25(6), 360-367.[↩]
  15. Gauer, R., & Braun, M. M. (2012). Thrombocytopenia. American family physician, 85(6), 612-622.[↩]
  16. Izak, M., & Bussel, J. B. (2014). Management of thrombocytopenia. F1000prime reports, 6.[↩]
  17. Winningham ML, MacVicar MG, Burke CA. Exercise for Cancer Patients: Guidelines and 382 Precautions. The Physician and sportsmedicine. 1986;14(10):125-134.[↩]
  18. Elter T, Stipanov M, Heuser E, et al. Is physical exercise possible in patients with critical cytopenia undergoing intensive chemotherapy for acute leukaemia or aggressive lymphoma? Int J Hematol. 2009;90(2):199-204.[↩]
  19. Ibanez, K., Espiritu, N., Souverain, R. L., Stimler, L., Ward, L., Riedel, E. R., … & Stubblefield, M. D. (2018). Safety and feasibility of rehabilitation interventions in children undergoing hematopoietic stem cell transplant with thrombocytopenia. Archives of physical medicine and rehabilitation, 99(2), 226-233.[↩]
  20. Crownover, B. K., & Covey, C. J. (2013). Hereditary hemochromatosis. American family physician, 87(3), 183-190.[↩]
  21. Iron Disorders Institute. http://www.irondisorders.org/. Accessed November 18, 2011.[↩]
  22. Peterson, C., & Goodman, C. C. (2015). The hematologic system. In C. C. Goodman & K. S. Fuller (Eds.), Pathology implications for the physical therapist (4th ed., pp. 705–771). St. Louis: Elsevier/Saunders.[↩]
  23. Allen, M. K. (2010). Hereditary hemochromatosis: a literature review and case report. Physiotherapy Canada, 62(3), 276-284.[↩]
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