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 .
Specific manifestations include the following:
- Anemia manifests as pale skin, palpitations, dyspnea, etc.
- Leukopenia results as frequent, reccurent infections and fever
- Thrombocytopenia causes easy bruising, nose bleeds, bleeding of gums and unexplained bleeding of the internal organs
- Easy Bruising
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]
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]
In addition to fatigue and easy bruising, the patient reports a history of unexplained lower extremity swelling and recurrent herpes infections. [hematology.org]
Affected patients may complain of fatigue, easy and prolonged bleeding, easy bruises, frequent infections, and abdominal discomfort. The vast majority of patients with HCL have splenomegaly (prevalence ranges from 66% to 100%) [8–11]. [hindawi.com]
Entire Body System
Figure 1: (a) 6yr/female diagnosed as Aplastic anemia. Bone marrow aspiration, (b) 6yr/female diagnosed as Aplastic anemia. Bone marrow biopsy Click here to view Figure 2: 35yr/male diagnosed as megaloblastic anemia. [mjdrdypu.org]
neutropenia Consider appropriate consults (e.g. heme/onc, infectious disease) Disposition See Also Anemia Thrombocytopenia Neutropenia, Leukopenia Immunocompromised antibiotics Aplastic anemia External Links References [wikem.org]
The commonest cause for pancytopenia was megaloblastic anemia (74.04%), followed by aplastic anemia (18.26%). [jlponline.org]
Key Words: Aplastic Anemia, Bone Marrow Biopsy, Megaloblastic Anemia, Pancytopenia INTRODUCTION Pancytopenia is the simultaneous presence of anemia, leucopenia and thrombocytopenia. [ncbi.nlm.nih.gov]
Dimorphic anemia (46%) was the predominant blood picture followed by macrocytic anemia. The commonest bone marrow finding was megaloblastic erythroid hyperplasia. [jmscr.igmpublication.org]
- Refractory Anemia
Bone marrow aspiration revealed refractory anemia with ringed sideroblasts of myelodysplastic syndrome (MDS). We diagnosed the case as copper deficiency-induced pancytopenia and ESA hyporesponsiveness related to zinc excess. [jstage.jst.go.jp]
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]
It has been suggested that some of these cases may just simply be cases of refractory anemia. [mjmsr.net]
We also found a single (0.4%) case of pancytopenia due to MDS of Refractory Anemia with Excessive Blasts (RAEB) type. The incidence of MDS as reported in other similar studies varies from 0 to 18% (Table 2). [bmchematol.biomedcentral.com]
- Chronic Infection
Similar studies showed the rate of pancytopenia due to severe chronic infections to range between 4 – 9% [3, 6, 8], but the details of the various types of infections was not available for comparison. [bmchematol.biomedcentral.com]
Background Anemia + Thrombocytopenia + Leukopenia Clinical Features Signs/symptoms of anemia (e.g. fatigue, dyspnea, tachycardia, pallor) Signs/symptoms of thrombocytopenia (e.g. petechiae/purpura, mucosal bleeding, GI bleed, hematuria, intracranial hemorrhage [wikem.org]
Because red blood cells carry oxygen to cells, a reduction in the number of red blood cells causes shortness of breath and rapid heartbeat, or tachycardia. [study.com]
Case: A 51-year old woman was admitted to the Hospital of Lithuanian University of Health Sciences, Kaunas clinics with 6 months history of tachycardia, bilateral leg oedema and progressive dyspnea. [endocrine-abstracts.org]
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 [symptoma.com]
Jaw & Teeth
- 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]
Medical Conditions Different medical syndromes like malignant osteoporosis, lymphohistiocytosis, dyskeratosis congenita and leishmaniasis are also considered to be causes for this disorder. [primehealthchannel.com]
Her past medical history is notable for rheumatoid arthritis being treated with methotrexate, as well as hypothyroidism, osteoporosis, and hypertension. In 2009, while on methotrexate, she experienced epistaxis, and was found to be pancytopenic. [healio.com]
- Bone Disorder
BRA Institute Rotary Cancer Hospital (AIIMS), 110029 - New Delhi/IN 3 Pathology, All India Institute of Medical Sciences, 110029 - New Delhi/IN Abstract Case Summary INTRODUCTION Osteopetrosis, is a dysplastic bone disorders due to dysfunctional osteoclast [oncologypro.esmo.org]
These problems include poor coordination and balance (ataxia), difficulty with movements that involve judging distance or scale (dysmetria), uncontrollable muscle contractions (clonus), and involuntary back-and-forth eye movements (nystagmus). [ghr.nlm.nih.gov]
She denied having fever, cough, chest pain, or vertigo. [mayoclinicproceedings.org]
To diagnose pancytopenia, bone marrow aspiration and biopsy are often necessary . 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 .
KEYWORDS: G6PC3; congenital neutropenia; lymphopenia; variable bone marrow phenotype [ncbi.nlm.nih.gov]
Chapter 101 Pancytopenia and febrile neutropenia First published: 06 May 2017 Summary Pancytopenia may be due to inadequate or defective production of blood cells (bone marrow failure), increased destruction or complex mechanisms. [onlinelibrary.wiley.com]
Management See management of anemia, thrombocytopenia, neutropenia Consider appropriate consults (e.g. heme/onc, infectious disease) Disposition See Also Anemia Thrombocytopenia Neutropenia, Leukopenia Immunocompromised antibiotics Aplastic anemia External [wikem.org]
- Decreased Platelet Count
Though iron deficiency is associated with a reactive thrombocytosis, increasing severity of iron deficiency leads to normalisation and occasionally even decrease platelet counts. [njlm.net]
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 .
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% .
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 . 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 .
Epidemiological studies have shown that the annual incidence of aplastic anemia in Europe is 2 cases per million population . In Asia, the incidence is higher . 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).
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 .
Pancytopenia is sometimes hereditary, and it is not possible to prevent it . 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).
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.
- 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.
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