Pancytopenia is defined by a decrease in blood lines 3 on the data numérationformule:
– Decreased hemoglobin (<13 g / dL in men, 12 g / dL in women);
– Decrease in neutrophils (<1,5.109 / L);
– Low platelets (<150,109 / L).
Diagnosis of pancytopenia:
The diagnosis is suspected in: – an anemic syndrome (pallor, fatigue, exertional dyspnea or tachypnea, tachycardia effort or rest);
– An infectious syndrome bacterial infections or severe mundane repetition does not normally responsive to antibiotic therapy, mouth ulceration)
– Hemorrhagic syndrome: petechial purpura, spontaneous bruising, recurrent epistaxis, buccal gingival bleeding or spontaneous bubbles. These signs may be absent, isolated or associated. The diagnosis can be made during a routine blood count (preoperative, health check). In most cases a line is more heavily impaired than others.
Assessing the severity of pancytopenia:
The severity will be evaluated primarily on the clinical findings and the results of the blood count. The severity of clinical signs are:
– Marked anemic syndrome dyspnea rest angina, fainting, mental retardation;
– Serious infectious syndrome: septicemic condition, signs of shock, cellulitis, severe hypoxic lung disease;
– Severe ulcerative syndrome extensive purpura, hemorrhagic bullae oral, gastrointestinal bleeding, sudden onset of headache, motor or sensory deficit suggestive of cerebral subarachnoid hemorrhage, hemorrhage of the fundus.Biological signs of severity are defined by the following criteria:
– Hemoglobin <8 g / dL;
– Neutrophils <0,5.109 / L;
– Platelets <20,109 / L.
A- Examination and clinical examination:
It is essential to clarify the presentation of symptoms when they exist: evolution-it is acute, subacute or chronic? The patient does dispose- previous blood counts, normal or pathological already? It is necessary to search for all drug intake emphasizing recent changes (sulfonamides, nonsteroidal anti-inflammatory drugs). The risks of toxic exposures, professional or not, will be evaluated. Making contact with the company occupational physician is sometimes essential. Signs of autoimmune diseases in history Wanted: arthralgia, rash, joint deformities. We must question the patient on all of its history, its past and current diseases and search these elements on its health book.
2- Clinical examination:
He immediately search splenomegaly (common cause of pancytopenia), hepatomegaly, lymphadenopathy, signs of portal hypertension. Signs suggestive of autoimmune diseases will also be sought carefully rash, joint deformities.
B- Laboratory tests:
The blood count abnormalities 3 research lines.
1- anomalies erythroid:
The number of reticulocytes to orient towards a peripheral mechanism when it is increased (120 109 / L). It is therefore necessary to prescribe a reticulocyte count in addition to the standard formula counts. If anemia is macrocytic, macrocytosis is generally moderate (98 μ3 or fl <mean corpuscular volume <110 μ3 or fl) and can be due to myelodysplasia, alcoholism, or high regeneration (high reticulocytosis ). A significant macrocytosis (> 110 μ3 or fl) makes suspect a vitamin B12 deficiency or folic acid. Other anemias encountered in pancytopenia are usually normocytic (normal average cell volume). A study of the morphology of red blood cells on a slide can highlight distortions evoking myelofibrosis (bone marrow fibrosis) in red blood cells drops, tears or pear. Highlighting schistocytes evokes a peripheral destruction.
2- Disorders of leukocyte lineage:
Circulating pathological cells can be detected by a trained eye (not highlighted by the controller): circulating blasts in the case of acute leukemia leucopenic shape, hairy in hairy cell leukemia.
3- Abnormal platelet lineage:
Increased volume of platelets evoke a peripheral origin.
C- medullary Reviews:
This is the key examination to differentiate central causes peripheral causes and determining the etiology of pancytopenia in most cases.
• The marrow is rich with:
– A maturation block: the case of myelodysplasia and acute leukemia;
– Invasion: it is also the case of acute leukemia but also of myeloma with plasma cell infiltration, lymphomas and metastatic medullary cancer;
– Megaloblastosis deficiencies in vitamin B12 or folate (which must be measured in the blood).
• The cord is poor in aplastic anemia often with a relative excess of lymphocytes.
• The cord is desert with a difficult puncture as in medullary fibrosis. A poor or desert marrow requires to perform a bone marrow biopsy quickly.
• The cord is normal, no abnormal maturation and thus sign a cause device.
2- marrow biopsy:
It is an indispensable instrument to confirm the diagnosis in cases of poor or desert marrow; it is often performed in the staging other situations (leukemia, lymphoma, myeloma, metastatic cancer). If circumstances beyond thrombocytopenia, it is desirable to transfuse platelets prior to the biopsy will after local anesthesia at the posterior superior iliac spine or earlier.
Additional examinations, particularly spinal cord, will help differentiate between pancytopenia central cause of peripheral causes.
1- Device Causes:
They are often associated with splenomegaly.
• This is the case in hypersplénismes, whatever the cause, with a moderate pancytopenia but sometimes with isolated cytopenia without clinical impact. Liver disease associated with portal hypertension is often the case, hence the need to carry out a gastroscopy in search of esophageal varices and an abdominal ultrasound with Doppler ultrasonography. With fever in a patient exposed (Mediterranean in particular), it is necessary to think and ask leishmaniasis serology as well as research on bone marrow smears of Leishmania in case of doubt or in immunocompromised patients, and usefulness of culture of gene amplification by PCR (polymerase chain reaction).
• In the absence of splenomegaly, think to portal hypertension of cirrhosis to be confirmed by percutaneous or transjugular liver biopsy if hemostasis allows.
• The immunological mechanism of pancytopenia are possible but rare, the most common bicytopénies in autoimmune diseases, including lupus with leukoneutropenia, hemolytic anemia and IgG anti-Rh (or) peripheral thrombocytopenia.
2- central Causes:
These causes are varied.
• Deficiencies in vitamin B12 or folate show a rich marrow megaloblastic look and blue after staining. Measurements of serum vitamin B12 and folic acid serum and erythrocyte will be requested.
• The myelodysplastic syndromes are diagnosed by myelogram, complemented by Perls staining and marrow cytogenetic examination to eventually end clonal abnormalities acquired. The bone marrow aspiration, the reaction Perls and cytochemistry make the difference between: sideroblastic anemia, refractory anemia, refractory anemia with excess blasts.
• The marrow is infiltrated by pathological elements in the case of acute leukemia, lymphomas, myeloma and spinal metastases. The cell characteristics are specified by the cell immunophenotype.
• Hairy cell leukemia often associated splenomegaly and pancytopenia. Bone marrow fibrosis is a common and essential bone marrow biopsy.
• The reticulin or collagen myelofibrosis may be associated with:
– Myeloid splenomegaly;
– Acute myelofibrosis;
– Spinal metastasis of cancer.
• Bone marrow suppression is affirmed bone marrow biopsy; it can be constitutional or acquired. The constitutional forms are seen in children (Fanconi anemia, dyskeratosis congenita). For acquired aplastic they are secondary idiopathic. In many cases, no one factor is highlighted and this is called idiopathic aplastic anemia. Among the secondary causes are: ionizing radiation, cancer chemotherapy (frequent cause obvious but that does not require investigating etiological), non-toxic drug exposures (use of adhesives and paints, benzene, solvents), the medications (nonsteroidal antiinflammatory, gold salts, D-penicillamine, sulfonamides, chloramphenicol, beta-lactams, furosemide, salicylates, ticlopidine, phenothiazines …). We should also mention the post-hepatitis aplasia. • paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli disease) may present as bone marrow suppression or as pancytopenia including medullary wealth varies. Anemia is often associated with iron deficiency with hemolytic component. Thrombosis associated or abdominal pain are valuable elements for the diagnosis is confirmed by flow cytometry of blood cells, particularly with CD59 and CD55 deficiency. The classic test of Ham-Dacia has lost much of its value since the development of these techniques. Finally, there are boundaries between forms aplastic anemia, myelodysplasia, and paroxysmal nocturnal hemoglobinuria.
Highlights to include:
• Pancytopenia is defined by the decrease of all formed elements of blood to the blood count.
• Its causes are multiple. Pancytopenia may be of peripheral origin (by destruction or sequestration) or central origin by quantitative or qualitative deficit of hematopoiesis.
• Bone marrow is the key examination, supplemented in most cases by a bone marrow biopsy.
Strong Points to remember:
• The pancytopenia with clinical or biological signs of severity are medical emergencies.
• The bone marrow biopsy is often needed to confirm the diagnosis.
• Bone marrow cytogenetics is very useful to confirm the clonal nature and prognosis of certain etiologies.