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Pancytopenia

Pancytopenias

Pancytopenia is defined as lack of all types of blood cells. It includes deficiency of erythrocytes, leukocytes, platelets, and even reticulocytes. This blood cell disorder is seen in many hematological diseases, especially in megaloblastic anemia. Aplastic anemia is also a hematological disease characterized by bone marrow hypoplasia and pancytopenia [1].


Presentation

Specific manifestations include the following:

Systemic and general symptoms may include weakness, exhaustion and loss of appetite.

Easy Bruising
  • Leukopenia results as frequent, reccurent infections and fever Thrombocytopenia causes easy bruising, nose bleeds, bleeding of gums and unexplained bleeding of the internal organs Systemic and general symptoms may include weakness, exhaustion and loss[symptoma.com]
  • HIV infection should also be ruled out especially if the patient presents with pancytopenia and a combination of fatigue, easy bruising, spontaneous bleeding, fever, rigors.[info.isabelhealthcare.com]
  • In addition to fatigue and easy bruising, the patient reports a history of unexplained lower extremity swelling and recurrent herpes infections.[hematology.org]
  • The list of symptoms is long, and each can vary in frequency and severity: Weakness Fatigue Skin problems, such as rashes or easy bruising Pale skin Rapid heart rate Shortness of breath Bleeding problems, such as bleeding gums, nosebleeds or internal[stjude.org]
  • More severe pancytopenia can cause symptoms including: shortness of breath pale skin fatigue weakness fever dizziness easy bruising bleeding tiny purple spots on your skin, called petechiae larger purple spots on your skin, called purpura bleeding gums[healthline.com]
Anemia
  • Nutritional deficiency related anemia is often seen in developing countries. In this study, we describe seven cases of severe thrombocytopenia, anemia and leukopenia (pancytopenia) from the same geographic locality and similar clinical presentation.[ncbi.nlm.nih.gov]
  • CONCLUSION: Anti-C(w) may lead to severe fetal anemia and consecutive complications.[ncbi.nlm.nih.gov]
  • This blood cell disorder is seen in many hematological diseases, especially in megaloblastic anemia. Aplastic anemia is also a hematological disease characterized by bone marrow hypoplasia and pancytopenia.[symptoma.com]
  • Pancytopenia usually requires a bone marrow biopsy in order to distinguish among different causes. anemia: hemoglobin 13.5 g/dL (male) or 12 g/dL (female). leukopenia: total white cell count 4.0 x 109/L.[en.wikipedia.org]
Refractory Anemia
  • Peripheral blood flow cytometry to look for PNH should be performed on all patients with aplastic anemia, suspected PNH, and certain forms of MDS, including hypoplastic MDS and some refractory anemia patients.[cancertherapyadvisor.com]
Heat Intolerance
  • A 27-year-old male patient who presented to the emergency room with complaints of sweating, palpitations, heat intolerance, insomnia and weight loss for the last 3 months. His medical history was significant for hypertension.[ncbi.nlm.nih.gov]
Bleeding Gums
  • Symptoms at presentation included: oral mucositis (n 37); fever (n 24); diarrhea (n 12), bleeding gums (n 5) and purpura (n 3).[ncbi.nlm.nih.gov]
  • The list of symptoms is long, and each can vary in frequency and severity: Weakness Fatigue Skin problems, such as rashes or easy bruising Pale skin Rapid heart rate Shortness of breath Bleeding problems, such as bleeding gums, nosebleeds or internal[stjude.org]
  • More severe pancytopenia can cause symptoms including: shortness of breath pale skin fatigue weakness fever dizziness easy bruising bleeding tiny purple spots on your skin, called petechiae larger purple spots on your skin, called purpura bleeding gums[healthline.com]
  • The following are common signs and symptoms attributable to thrombocytopenia: epistaxis, bleeding gums, petechiae (usually best seen on the pretibial areas and posterior pharynx), heavy menstrual bleeding, gastrointestinal bleeding, headache from intracranial[cancertherapyadvisor.com]
  • Gums are included ‡ Percentages not calculated because of different totals Table 3: Sensitivity, Specificity and Predictive Values for various laboratory findings in Diagnosis of Megaloblastic Anemia in 100 pancytopenic patients * Lab finding Sensitivity[ayubmed.edu.pk]
Tremor
  • On examination, he was tachycardic, hypertensive, had tremors of the upper extremities and a smooth goitre with a thyroid bruit.[ncbi.nlm.nih.gov]
  • CASE REPORT A 54 year-old female was referred to our hospital complaining of palpitations, hand tremor and a 23 kg weight loss in the last 5 months.[scielo.br]
Cerebellar Ataxia
  • Ataxia-pancytopenia (AP) syndrome is characterized by cerebellar ataxia, variable hematologic cytopenias, and predisposition to marrow failure and myeloid leukemia, sometimes associated with monosomy 7.[ncbi.nlm.nih.gov]
Meningism
  • Although meningitis is the commonest manifestation of cryptococcosis among HIV-infected patients, rare cutaneous manifestation with pancytopenia but with no meningeal signs indicate the HIV status in an endemic area of penicilliosis, Manipur.[ncbi.nlm.nih.gov]

Workup

To diagnose pancytopenia, bone marrow aspiration and biopsy are often necessary [8]. Bone marrow in patients with pancytopenia has fatty replacement and may show relatively increased non-hematopoetic elements. Consider dysplasia as well, and exclude the possibility of metastatic tumor.

In peripheral blood smear in pancytopenia is obvious paucity of all types of blood cells. Levels of hemoglobin and hematocrit are low, but level of hemoglobin in particular erythrocytes is around normal. Life duration of red blood cells is shorter than usual. Number of leukocytes is also reduced, especially number of granulocytes (granulocytes more than lymphocytes, that's why "relative lymphocytosis" occurs). The absolute numbers of platelets and reticulocytes are also reduced.

Many peripheral blood tests exist and may be useful, such as hemoglobin electrophoresis and blood-group testing, fluorescence-activated cell sorter (FACS) profiling, fluorescent-labeled inactive toxin aerolysin (FLAER) testing, histocompatibility testing, and many others [9].

Treatment

Pancytopenia can be treated differently. In the beginning of disease, but often not for long, blood transfusion may help to improve blood count. After a while, effectiveness of transfusion decreases. In most cases, patients are treated with drugs. If pancytopenia is suspected to be idiopathic and result of autoimmune response, the best solution is to use immunosuppressant drugs, such as corticosteroids, cyclosporine A, cyclophosphamide, thymoglobulin, and others.

In many cases, bone marrow-stimulators can be helpful. This group of drugs includes epoetin alfa, filgrastim and sargramostim. In not many cases, and among younger patients, bone marrow transplantation, or therapy with stem cells may be an option. The advantage of bone marrow transplantation compared to other kinds of treatment has been established in multicentric trials [10].

Prognosis

The outcome of patients with pancytopenia is improving because of better supportive care. The mortality is around 50% in first year after diagnosing aplastic anemia, without any treatment, but for patients that are receiving immunosuppressive treatment, 10-year survival rate is 68%, and for those who get hematopoietic cell transplantation is even higher, and amounts 73% [7].

Etiology

Pancytopenia can be idiopathic or secondary. In more than fifty percent of cases it is idiopathic, which means that the real cause of disorder is not actually known and detected [2]. It may be a response of body's immune system to its own substances.

In other cases, cause is well known and proven. It can be one of the physical agents, chemical agents or drugs, or the result of allergic reaction or infection. Also, sometimes pancytopenia occurs as result of bone marrow infiltration with fibrin, or lipoid or malignant cells. Sometimes, even a physiological condition as pregnancy may lead to pancytopenia, although it's very rare [3].

Epidemiology

Epidemiological studies have shown that the annual incidence of aplastic anemia in Europe is 2 cases per million population [4]. In Asia, the incidence is higher [5]. These studies have also shown that there is no racial or sexual predisposition for aplastic anemia. However, the incidence is slightly higher within younger patients (children and youth under age of 25).

Sex distribution
Age distribution

Pathophysiology

When there is insufficiency of bone marrow, it becomes fatty and empty in pluripotent hematopoetic stem cells. Still it is not defined what causes this insufficiency, and many theories tried to explain it. According to one theory, there is abnormality in structure of hematopoietic stem cells. In others, abnormality of hematopoietic microenvironment is suggested. Most likely, it is a result of immune response to different antigens, whether they are originally from outside or inside the organism [6].

Prevention

Pancytopenia is sometimes hereditary, and it is not possible to prevent it [11]. But in other cases, when the environmental factor is well known and detected, pancytopenia can be prevented by simple avoiding exposure (that is possible when cause is radiation, or use of drugs such as chloramphenicol, aspirin, mezanthion).

Summary

Erythrocytes have main role in oxygenating body's tissues, leukocytes defend the body against microorganisms and other antigens, and platelets are needed in hemostasis and blood coagulation. In pancytopenia, the disorder is expected in particular blood cell function. The symptoms are numerous and may include weakness, fatigue, frequent infections, bruising or spontaneous bleeding. Treatment can be various and depends on the etiology. Blood transfusion, medications such as corticosteroids, immunosuppressant drugs, bone marrow-stimulating drugs, stem-cell therapy, or bone marrow transplantation are common options. Also, sometimes removing some environmental factors may help.

Patient Information

  • Definition: Pancytopenia is deficiency of blood cells, including all types of cells (red, white and platelets). With bone marrow hypoplasia, it is main characteristic of some hematological diseases, such as aplastic anemia.
  • Cause: It can be caused by environmental factors, such as radiation, microorganisms (especially viruses), some particular drugs, and then it is secondary pancytopenia, because the cause is known. However, in most cases it is idiopathic, which means that cause is not known or detected. It could be the immune response to antigens, but that is just one of many pathophysiological theories.
  • Symptoms: The main symptoms are a result of blood cell function failure, and include tachycardia, weakness, pale skin (lack of red blood cells), frequent hyperthermia and infections (lack of white blood cells), unexplained bleeding, easy bruising (lack of platelets), and many others.
  • Diagnosis: Blood count and peripheral blood smear are necessary for diagnosing pancytopenia. In vitro analyzing cultures of hematopoietic tissue (myelogram) also can be useful. Bone marrow aspiration and biopsy are almost always required.
  • Treatment: There are several possibilities in treatment of pancytopenia - transfusion, immunosuppressive drugs and bone marrow-stimulating drugs, corticosteroids, antibiotics, bone marrow transplantation, and stem cell therapy.

References

Article

  1. Milosević R, Antonijević N, Janković G, Babić D, Colović M. Aplastic anemia - clinical characteristics and survival analysis. Serbian Arh Celok Lek. 1998 Jul-Aug;126(7-8):234-8.
  2. Das Makheja K, Kumar Maheshwari B, Arain S, Kumar S, Kumari S, Vikash.
  3. The common causes leading to pancytopenia in patient presenting to tertiary care hospital. Pak J Med Sci. 2013 Sep;29(5):1108-11.
  4. Zhang C, Liang MY, Wang SM. Clinical analysis of bicytopenia and pancytopenia during pregnancy. Zhonghua Fu Chan Ke Za Zhi. 2009 Jul;44(7):488-91. Chinese.
  5. Kaufman DW, Kelly JP, Levy M, Shapiro S. The Drug Etiology of Agranulocytosis and Aplastic Anemia. New York: Oxford University Press; 1991.
  6. Issaragrisil S, Sriratanasatavorn C, Piankijagum A, Vannasaeng S, Porapakkham Y, Leaverton PE, et al. Incidence of aplastic anemia in Bangkok. The Aplastic Anemia Study Group. Blood. May 15 1991;77(10):2166-8. 
  7. Taramura M, Miyoguchi H. Special Education: Aplastic anemia. 1996;1(3):187-189.
  8. Locasciulli A, Oneto R, Bacigalupo A, Socié G, Korthof E, Bekassy A, et al. Outcome of patients with acquired aplastic anemia given first line bone marrow transplantation or immunosuppressive treatment in the last decade: a report from the European Group for Blood and Marrow Transplantation (EBMT).Haematologica. Jan 2007;92(1):11-8.
  9. Jha A, Sayami G, Adhikari RC, Panta AD, Jha R.JNMA J Nepal Med Assoc. Bone marrow examination in cases of pancytopenia. 2008 Jan-Mar; 47(169):12-7.
  10. Krauss JS. The laboratory diagnosis of paroxysmal nocturnal hemoglobinuria (PNH): update 2010. Lab Medicine. 2012;43:20-4.
  11. Passweg JR, Pérez WS, Eapen M, Camitta BM, Gluckman E, Hinterberger W, et al. Bone marrow transplants from mismatched related and unrelated donors for severe aplastic anemia. Bone Marrow Transplant. Apr 2006;37(7):641-9.

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Last updated: 2017-08-09 17:20