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Acute Lymphoblastic Leukemia

ALL

Acute lymphocytic leukemia (ALL), otherwise referred to as acute lymphoblastic leukemia or acute lymphoid leukemia, is a hematopoietic malignancy that involves the overproduction of cancerous white blood cells in the bone marrow.

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Presentation

Acute lymphoblastic leukemia is a type of malignancy that most commonly affects children. The cancerous cells can either be found in the bone marrow itself, thus disrupting the process of blood cell production and maturation, or in other extramedullary organs.

The immature white blood cells that are produced by the diseased bone marrow initially replace the organ; as a result, it fails to produce the rest of the cellular lines, such as erythrocytes and platelets. Due to the pancytopenia that exists, patients can present with a multitude of symptoms. Specifically, a diminished erythrocyte count leads to anemia, pallor, weakness and fatigue, reduced platelets induce hemorrhagic events such as petechiae or active hemorrhage and the immature white blood cells cause an inadequate immune response to any infection. Although the patient is vulnerable to various microorganisms, they rarely exhibit septic phenomena. Many times, patients report a history of skeletal pain, arthritis and limping as the sole symptoms; as a result, diagnosis may be delayed [8]. Hepatosplenomegaly, fever and lymphadenopathy are also frequently observed.

In addition to the aforementioned symptoms, some types of ALL may lead to the development of masses outside of the borders of the bone marrow. The central nervous system, head and neck are the most common locations for these growths. The infiltration of the central nervous system may lead to papilledema, palsies of the cranial nerves, lethargy or nuchal rigidity; these symptoms rarely constitute the clinical manifestations at the time that ALL is originally diagnosed [9]. Another symptom that may raise suspicion of ALL is testicular edema in male patients that is not accompanied by pain.

Lastly, stridor is an alarming symptom that should indicate a possible mediastinal mass, which, in turn, may threaten the respiratory function. Corticosteroids should promptly be administered in order to prevent respiratory failure and close monitoring is also considered mandatory.

Easy Bruising
  • Symptoms of ALL include: Weakness or feeling tired Fever Easy bruising or bleeding Bleeding under the skin Shortness of breath Weight loss or loss of appetite Pain in the bones or stomach Pain or a feeling of fullness below the ribs Painless lumps in[nlm.nih.gov]
  • bruising or bleeding Petechiae (flat, pinpoint spots under the skin caused by bleeding) Shortness of breath Weight loss Loss of appetite Bone or joint pain Stomach pain Pain or a feeling of fullness below the ribs Painless lumps in the neck, underarms[uvahealth.com]
  • You should see your pediatrician if your child has any of the following: Unexplained fever and headaches Enlarged lymph nodes in the neck, arm pits, or groin Pain in the arms, legs, or back Easy bruising and bleeding Tiny red spots in the skin Fatigue[childrensnational.org]
  • Thrombocytopenia is a condition when there is a low number of platelets which can cause bleeding and easy bruising with no apparent cause. Low numbers of all three blood cell counts is called pancytopenia.[llscanada.org]
Splenomegaly
  • Splenomegaly and thrombocytopenia were frequently observed more in CD200 patients. Increased frequency of CD34 was associated with CD200 and CD56 patients.[ncbi.nlm.nih.gov]
  • Bone pain/tenderness, hepatomegaly, and splenomegaly may be present. About 7% of children have CNS involvement at the time of diagnosis.[lecturio.com]
  • Hepatomegaly and/or splenomegaly due to leukemic infiltration are present in many patients at diagnosis but are often overlooked.[clinicaladvisor.com]
  • Lymphadenopathy, splenomegaly, and hepatomegaly is seen in about 20% of patients with ALL. Abdominal masses are uncommon and if present suggest mature B-cell ALL (Burkitt's lymphoma/leukemia).[cancertherapyadvisor.com]
  • Left upper quadrant fullness and early satiety due to splenomegaly (10-20%). Dyspnoea (due to anaemia or large mediastinal mass in those with T-cell tumours).[patient.info]
Dyspnea
  • CASE REPORT We report the case of a 58-year-old male patient who presented with severe chest pain, dyspnea, systemic symptoms, leukopenia, normocytic anemia, and severe lactic acidosis.[ncbi.nlm.nih.gov]
  • But the patient also suffered from pulmonary infection, including fever and dyspnea. The patient was newly diagnosed with Ph ALL with pulmonary infection. The patient received oral dasatinib monotherapy (100 mg qd) for 28 days as induction therapy.[ncbi.nlm.nih.gov]
  • Dyspnea (difficulty breathing) — In some cases of ALL, leukemia cells tend to clump together and form a mass in the middle of the chest. This chest mass can cause pain and difficulty breathing in your child.[danafarberbostonchildrens.org]
  • In addition, patients can present with symptoms resulting from anemia, such as dyspnea or lightheadedness, infections due to neutropenia, or easy bruisability or bleeding resulting from thrombocytopenia.[cancertherapyadvisor.com]
  • Symptoms of anemia are common and include fatigue, dizziness, palpitations, and dyspnea upon even mild exertion. Other patients present with signs of bleeding. Bleeding can be the result of thrombocytopenia due to marrow replacement.[emedicine.medscape.com]
Cough
  • Wheezing, coughing and/or painful breathing requires immediate medical attention. ALL can cause a variety of symptoms in children based on their age and their disease type.[danafarberbostonchildrens.org]
  • Any wheezing, coughing and/or labored or painful breathing requires immediate medical attention.[chop.edu]
  • Coughing or needing to lean forward to breathe or to sleep upright are particularly ominous signs. Patients should not be set in the supine position, and sedation could lead to fatal cardiorespiratory collapse.[clinicaladvisor.com]
Fever
  • We present the case of a 10-year-old boy with acute lymphoblastic leukemia who developed a diffuse, morbilliform eruption in the setting of fever and pancytopenia.[ncbi.nlm.nih.gov]
  • A 6-year-old girl presented with pancytopenia, fever, and myelodysplasia. Following transient remission pre-B ALL was diagnosed 14 months later. Clonal B-lineage blasts at the period of pancytopenia were identified retrospectively.[ncbi.nlm.nih.gov]
  • Here, a 16-year-old male presented with complaints of pain abdomen, on and off fever, joint pain, and hepatosplenomegaly for 2 months. Bone marrow examination was suggestive of acute leukemia with numerous leukoblasts on aspiration.[ncbi.nlm.nih.gov]
  • We report a case of a 3 years old boy who presented with prolonged fever, nausea, vomiting and increasing lower limbs pain. Skeletal X-rays and CT scan showed severe osteolytic lesions of the skull and extremities.[ncbi.nlm.nih.gov]
  • But the patient also suffered from pulmonary infection, including fever and dyspnea. The patient was newly diagnosed with Ph ALL with pulmonary infection. The patient received oral dasatinib monotherapy (100 mg qd) for 28 days as induction therapy.[ncbi.nlm.nih.gov]
Anemia
  • Laboratory findings were remarkable for anemia, high transaminase levels, and high blast count. Histopathologic findings were consistent with early pernio. Further examination revealed acute B-cell lymphoblastic leukemia.[ncbi.nlm.nih.gov]
  • CASE REPORT We report the case of a 58-year-old male patient who presented with severe chest pain, dyspnea, systemic symptoms, leukopenia, normocytic anemia, and severe lactic acidosis.[ncbi.nlm.nih.gov]
  • Laboratory findings revealed severe hypercalcemia, anemia, and renal insufficiency with decreased serum parathyroid hormone. FDG PET/CT was performed for characteristics suggestive of multiple myeloma and other occult malignancy.[ncbi.nlm.nih.gov]
  • Laboratory tests are expected to reveal anemia and thrombocytopenia, alongside leukocytosis. Lymphoblasts may also be present.[symptoma.com]
  • The most common signs and symptoms of acute lymphoblastic leukemia in children are: Anemia. Anemia occurs when normal red blood cells can't be produced because the bone marrow is overcrowded by leukemia cells.[chop.edu]
Fatigue
  • A 21-year-old woman who presented fatigue had a new diagnosis of acute lymphoblastic leukemia underwent FDG PET/CT. The images demonstrated extramedullary infiltration in multiple organs, including the liver, pancreas, kidney and the bone.[ncbi.nlm.nih.gov]
  • We present the case of a 16-year-old boy who presented with fatigue, polyuria, and polydipsia while on chemotherapy for his relapsed acute lymphoblastic leukemia (ALL).[ncbi.nlm.nih.gov]
  • Medical Definition of acute lymphoblastic leukemia : lymphocytic leukemia that is marked by an abnormal increase in the number of lymphoblasts, that is characterized by rapid onset and progression of symptoms which include fever, anemia, pallor, fatigue[merriam-webster.com]
  • She was becoming easily fatigued and uncharacteristically cranky. He also thought she seemed to be bruising from only minor trauma. Her exam reveals cervical and axillary lymphadenopathy.[step2.medbullets.com]
  • Normally, these cells are relatively rare, but in ALL, they continuously multiply and are overproduced by the bone marrow, causing fatigue, anemia, fever, and bone pain due to the spread of these cells into the bone and joint surfaces.[medcell.med.yale.edu]
Weakness
  • A 19-year-old man presented with weakness, polydipsia, and polyuria for 1 month. NDI was diagnosed with insignificant response to a water deprivation test after stimulation with vasopressin injection.[ncbi.nlm.nih.gov]
  • Muscle weakness is one of the most serious problems during chemotherapy for childhood hematological malignancies. It may be caused by long-term hospitalization, unfavorable physical conditions, and restricted activities.[ncbi.nlm.nih.gov]
  • In patients with B-cell precursor-ALL (BCP-ALL), the high activity of the pathway correlates with the weak response to anti-leukemic drugs and relapse as a result of downstream prosurvival pathway activation, such as nuclear factor kappa B (NF-κB).[ncbi.nlm.nih.gov]
  • Specifically, a diminished erythrocyte count leads to anemia, pallor, weakness and fatigue, reduced platelets induce hemorrhagic events such as petechiae or active hemorrhage and the immature white blood cells cause an inadequate immune response to any[symptoma.com]
  • Symptoms of ALL include: Weakness or feeling tired Fever Easy bruising or bleeding Bleeding under the skin Shortness of breath Weight loss or loss of appetite Pain in the bones or stomach Pain or a feeling of fullness below the ribs Painless lumps in[nlm.nih.gov]
Weight Loss
  • Weight Loss Surgery is a Big Decision Our specialists provide the expertise and support you need to reach your weight loss goals.[uhhospitals.org]
  • Symptoms of ALL include: Weakness or feeling tired Fever Easy bruising or bleeding Bleeding under the skin Shortness of breath Weight loss or loss of appetite Pain in the bones or stomach Pain or a feeling of fullness below the ribs Painless lumps in[nlm.nih.gov]
  • Both leukemia itself and the treatment can lead to many problems such as bleeding, weight loss, and infections. Call your provider if you or your child develops symptoms of ALL.[nlm.nih.gov]
  • Possible Complications Both leukemia itself and the treatment can lead to many problems such as bleeding, weight loss, and infections. When to Contact a Medical Professional Call your provider if you or your child develops symptoms of ALL.[ufhealth.org]
Loss of Appetite
  • Symptoms of ALL include: Weakness or feeling tired Fever Easy bruising or bleeding Bleeding under the skin Shortness of breath Weight loss or loss of appetite Pain in the bones or stomach Pain or a feeling of fullness below the ribs Painless lumps in[nlm.nih.gov]
  • […] of appetite Enlarged lymph nodes The signs and symptoms of ALL can be the same as more common children’s illnesses and some children are treated for those other illnesses before leukemia is diagnosed.[curesearch.org]
  • […] of appetite Bone or joint pain Stomach pain Pain or a feeling of fullness below the ribs Painless lumps in the neck, underarms, stomach, or groin Swelling of the liver and/or spleen Diagnosis Your doctor will check for swelling of the liver, spleen or[uvahealth.com]
Vomiting
  • We report a case of a 3 years old boy who presented with prolonged fever, nausea, vomiting and increasing lower limbs pain. Skeletal X-rays and CT scan showed severe osteolytic lesions of the skull and extremities.[ncbi.nlm.nih.gov]
  • A 47-year-old man presented with abdominal pain, vomiting, and bone pain. Laboratory findings revealed severe hypercalcemia, anemia, and renal insufficiency with decreased serum parathyroid hormone.[ncbi.nlm.nih.gov]
  • […] cells are present, other symptoms may include: A full or swollen belly from leukemia cells in the liver or spleen Enlarged lymph nodes such as in the neck or groin, under arms, or above the collarbone Bone or joint pain Headache , trouble with balance, vomiting[webmd.com]
  • The cells sometimes – though not often – spread to other organs, and can cause more symptoms there: headaches, seizures and vomiting if the leukemia has spread to the brain or spinal cord, or difficulty breathing if it has spread to the chest cavity,[uihc.org]
Bleeding Gums
  • Symptoms include: Bone and joint pain Easy bruising and bleeding (such as bleeding gums, skin bleeding, nosebleeds, abnormal periods) Feeling weak or tired Fever Loss of appetite and weight loss Paleness Pain or feeling of fullness below the ribs from[nlm.nih.gov]
  • gums , or other unusual bleeding such as from minor cuts Depending upon where leukemia cells are present, other symptoms may include: A full or swollen belly from leukemia cells in the liver or spleen Enlarged lymph nodes such as in the neck or groin[webmd.com]
Palpitations
  • Symptoms of anemia are common and include fatigue, dizziness, palpitations, and dyspnea upon even mild exertion. Other patients present with signs of bleeding. Bleeding can be the result of thrombocytopenia due to marrow replacement.[emedicine.medscape.com]
  • Initial presentation is usually generalised fatigue and malaise but usually quickly progresses to bone marrow failure. [ 1 ] Symptoms Fatigue, dizziness and palpitations. Severe and unusual bone and joint pain.[patient.info]
  • Signs and symptoms Signs and symptoms of ALL include the following: Fever Signs and symptoms of anemia, such as pallor, fatigue, dizziness, palpitations, cardiac flow murmur, and dyspnea with even mild exertion Bleeding Blood clots Palpable lymphadenopathy[emedicine.medscape.com]
Tachycardia
  • The pallor, shortness of breath, tachycardia, and fatigue are due to anemia. Image : “Immature Large Blast Cells in Acute Lymphoblastic Leukemia.” by VashiDonsk.[lecturio.com]
  • Tachycardia and a flow murmur. Nonspecific signs of infection. Petechiae (due to thrombocytopenia), may progress into purpura or ecchymoses. Abdominal distention due to hepatomegaly and splenomegaly. Lymphadenopathy. Testicular enlargement.[patient.info]
Chest Pain
  • CASE REPORT We report the case of a 58-year-old male patient who presented with severe chest pain, dyspnea, systemic symptoms, leukopenia, normocytic anemia, and severe lactic acidosis.[ncbi.nlm.nih.gov]
  • Other symptoms may include swollen lymph nodes (glands), chest pain and abdominal discomfort due to a swollen spleen or liver. Occasionally people have no symptoms and ALL is diagnosed during a routine blood test.[leukaemia.org.au]
Heart Failure
Hepatosplenomegaly
  • Massive hepatosplenomegaly as a presentation of ALL has not been described previously in any patient population.[ncbi.nlm.nih.gov]
  • We report a 28-year-old male with the B-cell ALL who presented with extensive osteolytic lesions, bone pain, hepatosplenomegaly, and pancytopenia without circulating blasts in peripheral blood.[ncbi.nlm.nih.gov]
  • Here, a 16-year-old male presented with complaints of pain abdomen, on and off fever, joint pain, and hepatosplenomegaly for 2 months. Bone marrow examination was suggestive of acute leukemia with numerous leukoblasts on aspiration.[ncbi.nlm.nih.gov]
  • The most frequent signs are lymphadenopathies, hepatosplenomegaly, fever, signs of haemorrhage, and bone pain.[orpha.net]
Hepatomegaly
  • Bone pain/tenderness, hepatomegaly, and splenomegaly may be present. About 7% of children have CNS involvement at the time of diagnosis.[lecturio.com]
  • Lymphadenopathy, splenomegaly, and hepatomegaly is seen in about 20% of patients with ALL. Abdominal masses are uncommon and if present suggest mature B-cell ALL (Burkitt's lymphoma/leukemia).[cancertherapyadvisor.com]
  • Hepatomegaly and/or splenomegaly due to leukemic infiltration are present in many patients at diagnosis but are often overlooked.[clinicaladvisor.com]
  • Abdominal distention due to hepatomegaly and splenomegaly. Lymphadenopathy. Testicular enlargement. Gum hypertrophy. Leukaemia cutis. [ 9 ] Cranial nerve palsy (especially III, IV, VI and VIII) in mature B-cell ALL.[patient.info]
  • Datorita infiltratiei organelor cu celule leucemice pot apre splenomegalie, hepatomegalie si mai rar adenopatii. Rar pacientii pot prezenta rash tegumentar datorita infiltratiei tegumentului cu celule leucemice.[clinicaoncologieseverin.ro]
Bone Pain
  • We report a 28-year-old male with the B-cell ALL who presented with extensive osteolytic lesions, bone pain, hepatosplenomegaly, and pancytopenia without circulating blasts in peripheral blood.[ncbi.nlm.nih.gov]
  • A 47-year-old man presented with abdominal pain, vomiting, and bone pain. Laboratory findings revealed severe hypercalcemia, anemia, and renal insufficiency with decreased serum parathyroid hormone.[ncbi.nlm.nih.gov]
  • Normally, these cells are relatively rare, but in ALL, they continuously multiply and are overproduced by the bone marrow, causing fatigue, anemia, fever, and bone pain due to the spread of these cells into the bone and joint surfaces.[medcell.med.yale.edu]
  • The most frequent signs are lymphadenopathies, hepatosplenomegaly, fever, signs of haemorrhage, and bone pain.[orpha.net]
  • pain, sometimes associated with swelling of the joints The signs and symptoms of ALL can be the same as more common children’s illnesses and many children are treated for those other illnesses before leukemia is diagnosed.[childrensoncologygroup.org]
Arthralgia
  • Bone pain, arthralgia, and/or arthritis may manifest as decreased activity, general fussiness, irritability, or refusal to walk.[clinicaladvisor.com]
Petechiae
  • […] including X-rays and CT scans Previous chemotherapy or other treatment that weakens the immune system Symptoms of ALL You should see a doctor if you or your child is experiencing: Weakness Tiredness Fever Pale skin Night sweats Easy bruising or bleeding Petechiae[uvahealth.com]
  • Symptoms of ALL include: Frequent infections Fever Easy bruising Bleeding that is hard to stop Flat, dark-red skin spots (petechiae) due to bleeding under the skin Pain in the bones or joints Lumps in the neck, underarm, stomach or groin Pain or fullness[stjude.org]
  • […] bleeding (such as bleeding gums, skin bleeding, nosebleeds, abnormal periods) Feeling weak or tired Fever Loss of appetite and weight loss Paleness Pain or feeling of fullness below the ribs from an enlarged liver or spleen Pinpoint red spots on the skin (petechiae[nlm.nih.gov]
  • Symptoms of ALL include: Fever Easy bruising or bleeding that is hard to stop Flat, dark-red skin spots (petechiae) due to bleeding under the skin Pain in the bones or joints Lumps in the neck, underarm, stomach or groin Weakness, fatigue, paleness or[ochsner.org]
Night Sweats
  • ALL symptoms include: Weakness or feeling tired Fever or night sweats Shortness of breath Unusual bleeding or bruising Unexplained loss of appetite or weight loss Night sweats Bone or stomach pain Frequent infections Tiny, dark red spots under the skin[cancer.osu.edu]
  • sweats Easy bruising or bleeding Petechiae (flat, pinpoint spots under the skin caused by bleeding) Shortness of breath Weight loss Loss of appetite Bone or joint pain Stomach pain Pain or a feeling of fullness below the ribs Painless lumps in the neck[uvahealth.com]
  • sweats These symptoms can occur with other conditions.[nlm.nih.gov]
  • He had night sweats and no appetite. Thinking he had a virus, Elizabeth took Caleb to the emergency room. Within an hour, he was surrounded by doctors and nurses. “At that point it seemed like a bigger deal than a virus,” Elizabeth says.[mdanderson.org]
Purpura
  • The bleeding in ALL is due to decreased platelet counts and occurs in the skin and mucosal surfaces, manifesting as purpura and ecchymosis . Epistaxis, menorrhagia and gingival bleeding may also occur.[lecturio.com]
  • […] bleeding gums or nosebleeds high temperature (fever) of 38C (100.4F) or above night sweats bone and joint pain easily bruised skin swollen lymph nodes (glands) abdominal pain – caused by a swollen liver or spleen unexplained weight loss a purple skin rash (purpura[nhs.uk]
  • Petechiae (due to thrombocytopenia), may progress into purpura or ecchymoses. Abdominal distention due to hepatomegaly and splenomegaly. Lymphadenopathy. Testicular enlargement. Gum hypertrophy.[patient.info]
Epistaxis
  • A 30-year-old pregnant woman presented at 16 weeks' gestation with epistaxis, jaundice, diffuse abdominal pain and distension, massive hepatosplenomegaly and peripheral oedema.[ncbi.nlm.nih.gov]
  • Epistaxis, menorrhagia and gingival bleeding may also occur. The pallor, shortness of breath, tachycardia, and fatigue are due to anemia. Image : “Immature Large Blast Cells in Acute Lymphoblastic Leukemia.” by VashiDonsk.[lecturio.com]
Headache
  • […] do their jobs cause the symptoms that people have as the leukemic cells invade: fatigue, significant bruising (not just a few bumps and bruises on the shins), fever, significant swollen lymph nodes throughout the body, and less commonly bone pain or headache[hollywoodlife.com]
  • […] effects of Besponsa include low levels of platelets (thrombocytopenia), low levels of certain white blood cells (neutropenia, leukopenia), infection, low levels of red blood cells (anemia), fatigue, severe bleeding (hemorrhage), fever (pyrexia), nausea, headache[fda.gov]
  • You should see your pediatrician if your child has any of the following: Unexplained fever and headaches Enlarged lymph nodes in the neck, arm pits, or groin Pain in the arms, legs, or back Easy bruising and bleeding Tiny red spots in the skin Fatigue[childrensnational.org]
  • Depending upon where leukemia cells are present, other symptoms may include: A full or swollen belly from leukemia cells in the liver or spleen Enlarged lymph nodes such as in the neck or groin, under arms, or above the collarbone Bone or joint pain Headache[webmd.com]
Confusion
  • Some patients present with leukostasis and clinical symptoms of impaired circulation such as a headache, confusion and respiratory distress.[lecturio.com]
  • Tremor, headache, and other mental status changes (eg, confusion) have been reported.[bloodjournal.org]
  • Children often have a more aggressive and dramatic presentation that may result in extreme pain in the extremities and joints and can be confused with arthritis.[cancertherapyadvisor.com]

Workup

The first step towards diagnosing acute lymphocytic leukemia includes blood tests. Laboratory tests are expected to reveal anemia and thrombocytopenia, alongside leukocytosis. Lymphoblasts may also be present. A bone marrow biopsy is also required in order for the diagnosis of ALL to be definitive: the bone marrow is aspirated and biopsized, in order to detect an infiltration by precursor cells and to differentiate between a T or B type leukemia.

Furthermore, imaging modalities can illustrate the extent of the malignancy. Plain radiographs or a computerized tomography scan can be used to eliminate or confirm suspicion that the disease has disseminated to the brain or spinal cord. Finally, a lumbar puncture can also detect cancerous precursor cells within the cerebrospinal fluid.

As soon as the diagnosis of ALL is established, HLA-typing is promptly carried out [10] [11].

Mediastinal Mass
  • Lastly, stridor is an alarming symptom that should indicate a possible mediastinal mass, which, in turn, may threaten the respiratory function.[symptoma.com]
  • In T-cell ALL, a chest X-ray may sometimes show mediastinal widening or mediastinal mass .[lecturio.com]
  • Imaging studies No other imaging studies other than chest radiography to evaluate for a mediastinal mass are routinely required in pediatric ALL.[emedicine.medscape.com]
  • mass Cause [ edit ] In ALL, lymphoid cell development stops at the lymphoblast (arrow), which are also overproduced The cancerous cell in ALL is the lymphoblast.[en.wikipedia.org]
  • Seventeen percent of patients demonstrated T-lineage ALL; one quarter of these coexpressed myeloid antigens. [ 11 ] T-lineage ALL was associated with younger age, male sex, presence of a mediastinal mass, higher WBC count and hemoglobin level, longer[emedicine.medscape.com]
Decreased Platelet Count
  • After 16 months, the results of routine blood tests showed reduced hemoglobin levels and decreased platelet counts. In addition, blast-like cells were found in a peripheral blood smear.[ncbi.nlm.nih.gov]
  • The bleeding in ALL is due to decreased platelet counts and occurs in the skin and mucosal surfaces, manifesting as purpura and ecchymosis . Epistaxis, menorrhagia and gingival bleeding may also occur.[lecturio.com]

Treatment

Acute lymphocytic leukemia is a type of malignancy that may affect various organs and is treated pharmacologically, by administrating chemotherapy.

Treatment is individualized depending on the type of all. In general, every therapeutic plan centers around three basic treatment stages:

  • Remission-induction phase
  • Intensification phase
  • Continuation therapy, reserved for those patients who exhibit residual disease

T-cell acute lymphocytic leukemia is treated with the addition of asparaginase and cyclophosphamide and mature B-cell acute leukemia requires a brief but intensive period of chemotherapy with additional methotrexate, cytarabine and cyclophosphamide.

Prognosis

Acute lymphocytic leukemia is considered a curable malignancy, with survival rates being exceptionally high during the past years. Although it progresses rapidly and will threaten the life of the patient, if left untreated, proper therapeutic actions are promising and generally effective.

Specifically, prognosis depends on a number of factors. Pediatric patients exhibit a greater recovery rate in comparison to adult individuals; amongst the adult group, patients that are diagnosed with ALL before the age of 50 also display a better prognosis. Studies have delineated that the remission rate for patients below the age of 30 is 90%, for patients between the ages of 30 and 60 the rate decreases to 80% and reaches its lowest number, 52%, when referring to patients who are older than 60 years of age [7].

Furthermore, patients who have a white blood cell count greater than 50,000 at the time of diagnosis are expected to respond better to treatment. The extent of the disease, including infiltration of other organs, involvement of the central nervous system etc, also affects prognosis in a negative way.

The exact subtype of ALL also makes a difference in the prognosis: patients with a T-cell acute lymphocytic leukemia recover at a bigger rate, compared to individuals with B-cell ALL. Additionally, individuals whose cancer evinces complete remission during the first 5 weeks since the initiation of treatment are seen in a more positive light, in contradistinction to patients whose remission requires longer periods of time or is never achieved.

Etiology

The exact etiologic mechanisms that underlie ALL have yet to be firmly established. There have been, however, observations that lead to the illustration of various factors that are believed to play a role in the causes.

A genetic background has been proposed, especially due to the 25% possibility of a monozygotic twin to develop the disease, once its sibling is diagnosed with it. ALL has been linked to various other pathologies, such as trisomy 21, Bloom syndrome, Klinefelter syndrome and others [1] [2] [3]. Furthermore, the exposure to radiation or energy from a nuclear explosion, the use of tobacco products and hair dyes, as well as jobs related to electricity are all believed to contribute to the onset of acute lymphocytic leukemia [4].

It is also believed that viruses can lead to the development of such a malignancy, even though this hypothesis has not been substantiated scientifically. Various polymorphisms in the metabolism of folate is another potential etiologic factor [5].

Epidemiology

Although ALL can be diagnosed in patients of all ages, children between the age of 0 to 14 are the ones most commonly affected by it. In fact, this type of cancer has been estimated to display a yearly incidence of approximately 4 cases per 100,000 children in the aforementioned age group [6]. This particular incidence is considered to be an average worldwide estimation.

The disease exhibits a predilection for Caucasian male individuals and its frequency peak is between 2 and 5 years old.

Sex distribution
Age distribution

Pathophysiology

Acute lymphocytic leukemia is a malignancy of the hematopoietic system, namely the bone marrow and, subsequently, of the blood. The bone marrow is a vital organ responsible for the production and maturation of all the lines of the blood's components: erythrocytes, white blood cells and platelets.

The bone marrow produces all three types of cells; after a differentiation process is completed within the organ, it releases mature cells in the circulation. In the case of ALL normal white blood cells fail to differentiate properly and immature precursor cells are released into the circulation. This developmental arrest in the life cycle of leukocytes implies that the immune system does not possess cells that are mature enough to fight infection and the patient is therefore severely immunocompromised. Furthermore, the precursor cells known as lymphoblasts infiltrate the bone marrow to such a degree that they replace the organ's elements and, as a result, it is incapable of producing red cells and platelets as well.

Prevention

The exact etiologic factors that underlie acute lymphocytic leukemia have not yet been clarified; it is therefore not possible to provide any strategic plan concerning its avoidance.

Summary

Acute lymphocytic leukemia (ALL) is a malignancy of the bone marrow. It affects the leukocytes, which are produced from the precursor cells, but fail to differentiate into mature white cells, because of an early arrest in their development.

The malignancy is defined as acute, due to the fact that it progresses quickly and, if left without the administration of the proper chemotherapeutic agents, it may threaten the life of the patient. Children are more commonly affected by this type of cancer than adults: a young age is amongst the positive prognostic factors with regard to the efficacy of the treatment.

The causes of acute lymphocytic leukemia are still not definitively established, although a certain genetic substrate has been proposed. Other factors that potentially contribute to the onset of the disease include exposure to chemicals, smoking and folate metabolism disruption.

ALL is diagnosed via a complete blood count and a bone marrow biopsy to definitively confirm the diagnosis. A plain radiograph or magnetic resonance imaging scan can be used to detect sites of potential dissemination, which are usually found in the brain or spinal cord. It is treated with chemotherapy and prognosis is generally positive.

Patient Information

Acute lymphocytic leukemia (ALL) is a type of cancer that affects the bone marrow, and, subsequently, the blood. It can occur at any age, but children are most commonly affected by it.

The bone marrow is a vital organ, that is responsible for the production and maturation of all of the cellular components of the blood: red blood cells, white blood cells and platelets. In cases of ALL, the white blood cells do not develop properly and experience a very early arrest in their maturation process. As a result, they are released into the circulation at an earlier developmental stage and are too immature to fight infections and diseases. Moreover, these immature cells, called lymphoblasts, progressively replace the material that the bone marrow is made of and lead to a decreased production of red blood cells and platelets as well.

Patients with ALL present with a variety of symptoms. Decreased concentrations of red blood cells lead to anemia, fatigue and weakness; less platelets imply a tendency towards hemorrhaging and the presence of immature white blood cells leads to the inability of the organism to protect itself against infections.

Acute lymphocytic leukemia is diagnosed with a complete blood count and a bone marrow biopsy. Radiologic evaluation is necessary to find out whether the malignant cells have infiltrated the brain or spinal cord. The condition is treated with chemotherapy.

References

Article

  1. De Keersmaecker K, Marynen P, Cools J. Genetic insights in the pathogenesis of T-cell acute lymphoblastic leukemia. Haematologica. 2005; 90:1116-1127.
  2. Machatschek JN, Schrauder A, Helm F, et al. Acute lymphoblastic leukemia and Klinefelter syndrome in children: two cases and review of the literature. Pediatr Hematol Oncol. 2004; 21:621-626.
  3. Greaves MF, Maia AT, Wiemels JL, et al. Leukemia in twins: lessons in natural history. Blood. 2003; 102:2321-2333.
  4. Snyder DS, Stein AS, O'Donnell MR, et al. Philadelphia chromosome-positive acute lymphoblastic leukemia secondary to chemoradiotherapy for Ewing sarcoma. Report of two cases and concise review of the literature. Am J Hematol. 2005; 78:74-78.
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  6. Ribera JM, Oriol A. Acute lymphoblastic leukemia in adolescents and young adults. Hematol Oncol Clin North Am. 2009 Oct; 23(5):1033-42, vi. 
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Last updated: 2018-06-21 20:04