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Chronic Lymphocytic Leukemia

B-CLL

Chronic lymphocytic leukemia (CLL) is the most common leukemia of adults in the Western world.

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Presentation

The average age of CLL patients is more than 70 years and it should be noted that a complex clinical picture occurs in more than half of the cases due to coexisting diseases. The CLL itself leads to unspecific symptoms such as general malaise and weakness, possibly also to fever, night sweats and weight loss. In the physical examination, generalized lymphadenopathy and hepato- and/or splenomegaly may be noted. The excessive proliferation of the lymphocytes results in immunological insufficiency and the patients are more susceptible to infections. Recurrent infections of the respiratory tract and urogenital tract are regularly described, and in fact pneumonia and sepsis are the most common causes of death associated with CLL. At the same time there are disturbances in the formation of erythrocytes and thrombocytes, leading to symptoms of anemia and hemorrhagic diathesis, patients are pale, their performance is reduced, they complain of headaches and dizziness as well as palpitations. Epistaxis, gastrointestinal bleeding, and to the formation of hematomas are also common. CLL patients also tend to autoimmune reactions. If these are directed against erythrocytes or blood platelets, the aforementioned symptoms are further intensified.

An acute increase in lymphadenopathy or organomegaly, as well as a worsening of the general condition with accentuation of B symptoms point to a Richter's transformation, a progression to an aggressive Non-Hodgkin lymphoma or - more rarely - to Hodgkin's disease.

Splenomegaly
  • Optimum therapy includes a combination of radiotherapy and chemotherapy, which may or may not be accompanied with surgery for existing complications such as a splenomegaly or hepatomegaly.[symptoma.com]
  • After 3 years, he presented a CLL progression with worsening lymphocytosis, anemia, thrombocytopenia, increased splenomegaly, and lymphadenopathies. Positron emission tomography-computed tomography scan excluded pathologic uptake.[ncbi.nlm.nih.gov]
  • […] the eyehole, of the base of the nasal pyramid, and of the left zygomatic region; anterior nasal bleeding; pustulous non-itching lesions at the cervical region and posterior thorax; enlarged bilateral axillary lymph nodes; hepatomegaly; and moderate splenomegaly[ncbi.nlm.nih.gov]
  • Imaging may identify various features of the disease such as splenomegaly , hepatomegaly /- lymphadenopathy , although these are not specific for the disease.[radiopaedia.org]
Generalized Lymphadenopathy
  • In the physical examination, generalized lymphadenopathy and hepato- and/or splenomegaly may be noted. The excessive proliferation of the lymphocytes results in immunological insufficiency and the patients are more susceptible to infections.[symptoma.com]
  • Clinical Information A chronic leukemia characterized by abnormal b-lymphocytes and often generalized lymphadenopathy.[icd9data.com]
  • Miranda , Composite Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma and T-Prolymphocytic Leukemia Presenting with Lymphocytosis, Skin Lesions, and Generalized Lymphadenopathy , Case Reports in Pathology , 10.1155/2019/4915086 , 2019 , (1-10) ,[doi.org]
  • Physical examination In addition to localized or generalized lymphadenopathy, patients may manifest the following: Splenomegaly (30-54% of cases) Hepatomegaly (10-20% of cases) Petechiae Pallor Nabhan C, Rosen ST.[emedicine.medscape.com]
Massive Splenomegaly
  • splenomegaly and occasionally lymphadenopathy; prognosis is often poor.[medical-dictionary.thefreedictionary.com]
  • Bulky disease was defined as the presence of lymph-nodes more than 5 cm in longest diameter by CT and/or ultrasound examination or the presence of massive splenomegaly (6 cm below the left costal margin).[mjhid.org]
  • Massive splenomegaly (  6 cm) resolved in 90% of responders and 25% overall.[doi.org]
Inguinal Lymphadenopathy
  • lymphadenopathy, spleen, liver) B: no anemia, no thrombocytopenia, 3 or more lymphoid areas enlarged C: anemia or thrombocytopenia Reference : U.S.[pathologyoutlines.com]
Cervical Lymphadenopathy
  • An 80-year-old man presented with painless mass of the right parotid gland of 2 years duration with recent ulceration of the overlying skin and right cervical lymphadenopathy underwent a surgical resection of parotid mass and biopsy of the periglandular[ncbi.nlm.nih.gov]
Fever
  • The symptoms of this infection range from a silent infection to fever, enlargement of the liver and spleen, weight loss, and pancytopenia.[ncbi.nlm.nih.gov]
  • Acute leukemias affect immature cells; the disease develops rapidly, with symptoms including anemia, fever, bleeding, and swelling of the lymph nodes.[britannica.com]
  • Common symptoms are enlarged lymph nodes, fever, night sweats, fatigue, weight loss and frequent infections.[symptoma.com]
  • CASE PRESENTATION: We present the case of a 73-year-old Caucasian patient, a rare case in which Wegener's granulomatosis is associated in a patient with chronic lymphocytic leukemia, who is admitted in the Infectious Disease Department for fever, diplopia[ncbi.nlm.nih.gov]
  • One third of the 224 patients had / one episode of infection, and 10% had a fever of unknown origin. FCR produced a high CR rate in previously untreated CLL. Most patients had no detectable disease on flow cytometry at the end of therapy.[ncbi.nlm.nih.gov]
Anemia
  • KEYWORDS: Autoimmune hemolytic anemia; Chronic lymphocytic leukemia; Ibrutinib [Indexed for MEDLINE] Free full text Publication type, MeSH terms, Substances Publication type Case Reports MeSH terms Aged Anemia, Hemolytic, Autoimmune/blood Anemia, Hemolytic[ncbi.nlm.nih.gov]
  • Anemia and thrombocytopenia are the major adverse prognostic variables. CLL has no standard staging system.[web.archive.org]
  • There is no anemia or thrombocytopenia. Clinical stage B: In this stage, 3 or more than 3 areas of lymphoid enlargement are found. There is no or little evidence of anemia or thrombocytopenia.[symptoma.com]
  • Group C: anemia (Hb less than 10 g) and/or thrombopenia (platelets less than 100,000/mm3); about 15% of the patients; median of 2 years.[ncbi.nlm.nih.gov]
  • , splenomegaly, or anemiaBinet classification Clinical stage A: characterized by no anemia or thrombocytopenia and fewer than three areas of lymphoid involvement (Rai stages 0, I, and II) Clinical stage B: characterized by no anemia or thrombocytopenia[en.wikipedia.org]
Lymphadenopathy
  • Hepatosplenomegaly and lymphadenopathy may or may not be present. Stage IV: Absolute lymphocytosis with thrombocytopenia ( 100,000/mm3). Lymphadenopathy, hepatosplenomegaly and anemia may or may not be present.[symptoma.com]
  • After 3 years, he presented a CLL progression with worsening lymphocytosis, anemia, thrombocytopenia, increased splenomegaly, and lymphadenopathies. Positron emission tomography-computed tomography scan excluded pathologic uptake.[ncbi.nlm.nih.gov]
  • Chronic lymphocytic leukemia–stages 0 Lymphocytosis in blood, without other Sx of leukemia I Lymphocytosis; lymphadenopathy II Lymphocytosis; lymphadenopathy; hepatosplenomegaly III Lymphocytosis; lymphadenopathy; hepatosplenomegaly, anemia IV Lymphocytosis[medical-dictionary.thefreedictionary.com]
  • An 80-year-old man presented with painless mass of the right parotid gland of 2 years duration with recent ulceration of the overlying skin and right cervical lymphadenopathy underwent a surgical resection of parotid mass and biopsy of the periglandular[ncbi.nlm.nih.gov]
  • On physical exam, he has notable tender lymphadenopathy. Peripheral smear reveals some spherocytes and lymphocytosis. His leukocyte count is 35,000/μL with 95% lymphocytes and a Hb of 7 g/dL. His direct Coombs test is positive with anti-IgG.[step2.medbullets.com]
Weight Loss
  • Common symptoms are enlarged lymph nodes, fever, night sweats, fatigue, weight loss and frequent infections.[symptoma.com]
  • The symptoms of this infection range from a silent infection to fever, enlargement of the liver and spleen, weight loss, and pancytopenia.[ncbi.nlm.nih.gov]
  • Weight Loss Surgery is a Big Decision Our specialists provide the expertise and support you need to reach your weight loss goals.[uhhospitals.org]
Fatigue
  • Call a provider if you develop enlarged lymph nodes or unexplained fatigue, bruising, excessive sweating, or weight loss.[nlm.nih.gov]
  • FINDINGS: Common adverse events were neutropenia, diarrhea, nausea, anemia, upper respiratory tract infection, thrombocytopenia, and fatigue.[ncbi.nlm.nih.gov]
  • CASE PRESENTATION: We describe a case of a 77-year-old man who presented with fatigue, pallor and lower limb pain and weakness. Initial laboratory studies showed Hb 7.7 g/dl, WBC 279.6   10(9)/1, PLT 143 10(9)/1.[ncbi.nlm.nih.gov]
  • Some patients notice fatigue, enlargement of lymph nodes or fullness in the abdomen due to an enlarged spleen.[ucsfhealth.org]
  • When to Contact a Medical Professional Call a provider if you develop enlarged lymph nodes or unexplained fatigue, bruising, excessive sweating, or weight loss.[mountsinai.org]
Pneumonia
  • OUTCOMES: Pneumonia and coagulopathy occurred after chemotherapy, and the patient died shortly thereafter. LESSONS: It is essential to recognize the transformation of disease earlier in chronic lymphoblastic leukemia patient.[ncbi.nlm.nih.gov]
  • […] with no history of travel outside the United States, who presented with chronic lymphocytic leukemia and S. stercoralis hyperinfection characterized by acute respiratory failure, altered mental status, and extended-spectrum-beta-lactamase Klebsiella pneumoniae[ncbi.nlm.nih.gov]
  • Bacterial infections such as skin infections, fluid and inflammation of the lungs (pneumonia), and inflammation of the sinuses (sinusitis) often occur. As the disorder advances, the patient loses the ability to fight off infections.[rarediseases.org]
  • One patient died due to pneumonia. Additionally, all patients experienced “rapid reductions in lymphadenopathy,” Byrd and colleagues said in the conference abstract.[curetoday.com]
Pleural Effusion
  • Effusion/complications Pleural Effusion/pathology* Remission Induction Thoracic Duct/diagnostic imaging Thoracic Duct/pathology* Treatment Outcome[ncbi.nlm.nih.gov]
Early Satiety
  • Symptoms and signs may be absent or may include lymphadenopathy, splenomegaly, hepatomegaly, fatigue, fevers, night sweats, unintentional weight loss, and early satiety. Diagnosis is by flow cytometry and immunophenotyping of peripheral blood.[merckmanuals.com]
  • If the spleen grows in size, a patient might feel bloated and have the sensation of early satiety (filling up with food after eating only a small amount). Learn more about CLL/SLL at cancer.net .[medicine.duke.edu]
  • satiety) Weight loss The health care provider will perform a physical exam and ask about your symptoms.[nlm.nih.gov]
  • satiety mucocutaneous bleeding from thrombocytopenia Physical exam painful lymphadenopathy hepatosplenomegaly Evaluation Labs – CBC WBC ( 20,000/μL) with 80% lymphocytes RBCs (attacked by CLL cells) PLTs (attacked by CLL cells) IgG in half of patients[step2.medbullets.com]
Abdominal Fullness
  • As the disease progresses, symptoms can include: enlarged lymph nodes tiredness fever night sweats weight loss frequent infections abdominal fullness Make an appointment with your doctor if you develop any of the above symptoms.[healthline.com]
  • fullness with early satiety, increased frequency of infections, and symptoms of an autoimmune cytopenia, as well as enlarged lymph nodes, hepatomegaly, and splenomegaly, which can be detected by palpation. 1 Essential components of the CLL diagnosis[targetedonc.com]
Palpitations
  • At the same time there are disturbances in the formation of erythrocytes and thrombocytes, leading to symptoms of anemia and hemorrhagic diathesis, patients are pale, their performance is reduced, they complain of headaches and dizziness as well as palpitations[symptoma.com]
  • […] any of these symptoms during or after an infusion of RITUXAN: Hives (red itchy welts) or rash Itching Swelling of your lips, tongue, throat, or face Sudden cough Shortness of breath, difficulty breathing, or wheezing Weakness Dizziness or feel faint Palpitations[rituxan.com]
Bleeding Gums
  • Thrombocytopenia A shortage of platelets, which can cause excess bruising and bleeding, such as frequent or severe nosebleeds or bleeding gums.[uihc.org]
Hepatomegaly
  • […] swelling of the left region of the eyehole, of the base of the nasal pyramid, and of the left zygomatic region; anterior nasal bleeding; pustulous non-itching lesions at the cervical region and posterior thorax; enlarged bilateral axillary lymph nodes; hepatomegaly[ncbi.nlm.nih.gov]
  • Imaging may identify various features of the disease such as splenomegaly , hepatomegaly /- lymphadenopathy , although these are not specific for the disease.[radiopaedia.org]
  • Optimum therapy includes a combination of radiotherapy and chemotherapy, which may or may not be accompanied with surgery for existing complications such as a splenomegaly or hepatomegaly.[symptoma.com]
  • Symptoms and signs may be absent or may include lymphadenopathy, splenomegaly, hepatomegaly, fatigue, fevers, night sweats, unintentional weight loss, and early satiety. Diagnosis is by flow cytometry and immunophenotyping of peripheral blood.[merckmanuals.com]
Hepatosplenomegaly
  • Chronic lymphocytic leukemia–stages 0 Lymphocytosis in blood, without other Sx of leukemia I Lymphocytosis; lymphadenopathy II Lymphocytosis; lymphadenopathy; hepatosplenomegaly III Lymphocytosis; lymphadenopathy; hepatosplenomegaly, anemia IV Lymphocytosis[medical-dictionary.thefreedictionary.com]
  • Hepatosplenomegaly and lymphadenopathy may or may not be present. Stage IV: Absolute lymphocytosis with thrombocytopenia ( 100,000/mm3). Lymphadenopathy, hepatosplenomegaly and anemia may or may not be present.[symptoma.com]
  • Stage I Stage I CLL is characterized by absolute lymphocytosis with lymphadenopathy without hepatosplenomegaly, anemia, or thrombocytopenia.[web.archive.org]
  • Stage I : Stage I CLL is characterized by absolute lymphocytosis with lymphadenopathy without hepatosplenomegaly, anemia, or thrombocytopenia.[texasoncology.com]
Night Sweats
  • Common symptoms are enlarged lymph nodes, fever, night sweats, fatigue, weight loss and frequent infections.[symptoma.com]
  • Symptoms of CLL may include: Abnormal bruising (if platelets are low) Enlarged lymph nodes , liver, or spleen Excessive sweating , night sweats Fatigue Fever Infections that keep coming back (recur) despite treatment Loss of appetite or becoming full[nlm.nih.gov]
  • Symptoms and signs may be absent or may include lymphadenopathy, splenomegaly, hepatomegaly, fatigue, fevers, night sweats, unintentional weight loss, and early satiety. Diagnosis is by flow cytometry and immunophenotyping of peripheral blood.[merckmanuals.com]
  • Other signs of CLL include: Fatigue Lethargy Night sweats Unexplained weight loss Unusual fullness after a meal Abdominal pain or swelling Fever and/or infection Bleeding Bruising[pennmedicine.org]
Petechiae
  • The most common treatment-related AEs (reported in at least 5 percent of patients) were headache (20 percent), an increased tendency to bruise (12 percent) and petechiae (12 percent).[curetoday.com]
  • Mucocutaneous bleeding and/or petechiae may be due to thrombocytopenia. Tiredness and fatigue may be present secondary to anemia; 10% of patients with CLL will present with an autoimmune hemolytic anemia .[emedicine.medscape.com]
  • Common leukemia symptoms are: persistent fatigue, weakness; frequent infections; weight loss without trying; swollen lymph nodes, enlarged liver or spleen; easy bleeding or bruising; tiny red spots in the skin (petechiae); and bone pain or tenderness.[publichealth.va.gov]
  • These are due to small superficial capillary bleeds known as petechiae (pronounced: pe-tee-chi-a), due to a very low platelet count. frequent or repeated infections and slow healing, due to a lack of normal white blood cells pain or discomfort under the[leukaemia.org.au]
  • Mucocutaneous bleeding and/or petechiae may be due to thrombocytopenia. unexplained weight loss Note: These signs and symptoms may also be caused by other conditions.[lymphomation.org]
Epistaxis
  • Epistaxis, gastrointestinal bleeding, and to the formation of hematomas are also common. CLL patients also tend to autoimmune reactions.[symptoma.com]
Headache
  • PRESENTATION: We present the case of a 73-year-old Caucasian patient, a rare case in which Wegener's granulomatosis is associated in a patient with chronic lymphocytic leukemia, who is admitted in the Infectious Disease Department for fever, diplopia, headache[ncbi.nlm.nih.gov]
  • The most common treatment-emergent AEs (reported in at least 20 percent of patients) were headache (43 percent), diarrhea (38 percent) and increased weight (25 percent).[curetoday.com]
  • […] or brown urine, unexpected bleeding or bleeding that is severe or that you cannot control, vomit blood or vomit looks like coffee grounds, cough up blood or blood clots, increased bruising, dizziness, weakness, confusion, change in your speech, or a headache[imbruvica.com]
  • At the same time there are disturbances in the formation of erythrocytes and thrombocytes, leading to symptoms of anemia and hemorrhagic diathesis, patients are pale, their performance is reduced, they complain of headaches and dizziness as well as palpitations[symptoma.com]
  • Tell your healthcare provider right away if you have any symptoms of infection: Fever Cold symptoms, such as runny nose or sore throat that do not go away Flu symptoms, such as cough, tiredness, and body aches Earache or headache Pain during urination[rituxan.com]
Dizziness
  • Tell your healthcare provider if you get any symptoms of heart rhythm problems, such as feeling as if your heart is beating fast and irregular, lightheadedness, dizziness, shortness of breath, chest discomfort, or you faint High blood pressure (hypertension[imbruvica.com]
  • Tell your healthcare provider right away if you have new or worsening symptoms or if anyone close to you notices these symptoms: Confusion Dizziness or loss of balance Difficulty walking or talking Decreased strength or weakness on one side of your body[rituxan.com]
  • At the same time there are disturbances in the formation of erythrocytes and thrombocytes, leading to symptoms of anemia and hemorrhagic diathesis, patients are pale, their performance is reduced, they complain of headaches and dizziness as well as palpitations[symptoma.com]
  • This shortage can cause the following symptoms: Fatigue Feeling week, or low energy Dizziness or lightheadedness Fever Enlarged lymph nodes Enlarged spleen, often feeling like pain or “fullness” in the belly Weight loss Night sweats Many signs of CLL[uihc.org]
  • Possible symptoms may include: anaemia, due to a lack of red cells causing persistent tiredness, dizziness, paleness, or shortness of breath when physically active increased or unexplained bleeding or bruising, and/or the appearance of red or purple flat[leukaemia.org.au]
Peripheral Neuropathy
  • I had bad peripheral neuropathy of the legs after using a small dose of vincristine in 2007; Dr. Droll said it was not an especially important part of the protocol and left it out.[clldiary.blogspot.com]

Workup

Work up includes a detailed history and physical examination.

Laboratory tests

  • Complete blood count
  • Blood profile
  • Lymph node aspiration
  • FISH
  • Array-based karyotyping
  • Matute's CLL Score
  • Flow cytometry

Imaging

  • Presence of characteristic 'smudge cells' on microscopic view
  • Bone marrow biopsy
  • CT scan/radiography to stage the disease
  • MRI

Test results

CLL is a diagnosed most commonly during routine physical examination and blood work. If the blood work points towards a leukemia, biopsy, cytometry and other such laboratory tests along with imaging studies should be performed to identify and stage the cancer.

Enlargement of the Spleen
  • The disease often involves enlargement of lymph nodes in various parts of the body as well as enlargement of the spleen.[texasoncology.com]
  • CLL leads to unspecific symptoms such as general malaise and weakness, fever, night sweats, weight loss and enlarged lymph nodes, spleen or liver.[symptoma.com]
  • Full medical history by your doctor Full physical exam with attention paid to enlarged liver and spleen Blood tests: Complete Blood Count (CBC) to check the number of white blood cells, red blood cells and platelets in your blood.[massgeneral.org]
  • […] of the spleen bone pain Chronic lymphocytic leukemia affects people in different ways.[medbroadcast.com]
Enlargement of the Liver
  • […] spleen or liver or both; stage III, lymphocytosis with anemia; and stage IV:lymphocytosis with thrombocytopenia.[ncbi.nlm.nih.gov]
  • The symptoms of this infection range from a silent infection to fever, enlargement of the liver and spleen, weight loss, and pancytopenia.[ncbi.nlm.nih.gov]
  • Symptoms of CLL may include: Abnormal bruising (if platelets are low) Enlarged lymph nodes , liver, or spleen Excessive sweating , night sweats Fatigue Fever Infections that keep coming back (recur) despite treatment Loss of appetite or becoming full[nlm.nih.gov]
  • Signs and symptoms include the following: Enlarged lymph nodes, liver, or spleen Recurring infections Loss of appetite or early satiety Abnormal bruising (late-stage symptom) Fatigue Night sweats See Presentation for more detail.[emedicine.medscape.com]
Lymphocytes Increased
  • Also, as the amount of lymphocytes increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may result in infection, anemia , and easy bleeding.[web.archive.org]
  • As the number of these abnormal lymphocytes increases, they interfere with the production of other important blood cells, leading to multiple complications, including infection, easy bleeding and swollen lymph nodes.[fredhutch.org]
  • Treatment may not be needed for years, and then only if the number of lymphocytes increases, the lymph nodes enlarge, or the number of red blood cells or platelets decreases.[medbroadcast.com]
Decreased Platelet Count
Pleural Effusion
  • Effusion/complications Pleural Effusion/pathology* Remission Induction Thoracic Duct/diagnostic imaging Thoracic Duct/pathology* Treatment Outcome[ncbi.nlm.nih.gov]

Treatment

Treatment is subjective to the stage of the disease. Since it does not have any definite cure, treatment focuses on improving the quality and quantity of life. Optimum therapy includes a combination of radiotherapy and chemotherapy, which may or may not be accompanied with surgery for existing complications such as a splenomegaly or hepatomegaly. Immunotherapy with antibodies against proteins found on the surface of CLL/SLL cells, such as CD20 and CD52, is finding increasing use [8].

Bone marrow transplant can be a potential cure but success rate is not a 100%. There is increasing interest in the use of reduced-intensity allogenic stem cell transplantation, which offers the prospect of cure for selective patients with a suitable donor [9].

Target therapy

Monoclonal antibody therapy and tyrosine-kinase inhibitor therapy are types of targeted therapy used in the treatment of chronic lymphocytic leukemia.

Prognosis

The overall median survival is about 6 years [3]. Prognosis depends upon the stage of cancer. If the patient is at Stage A/0, prognosis is the best, i.e 12-15 years with treatment. As the stage increases, prognosis of this disease worsens. In case of transformation of CLL into other, more aggressive cancers, prognosis is the worst. Both prolymphocytic and large-cell transformation are ominous events, with most patients surviving less than 1 year [7].

Etiology

CLL is a type of B cell lymphoma which presents primarily in the lymph nodes [3]. Much like other types of cancers, CLL has no definite known cause. It is an acquired disorder, and reports of truly familial cases are exceedingly rare [4]. However, genetic mutations and more commonly, deletions, may he held the culprit behind this disease.

4 main genetic aberrations are recognised in CLL cells that have a major impact on disease behaviour [5]. These include:

  • Deletion of 17p
  • Deletion of 13q (most common finding)
  • Trisomy 12 
  • Deletion of 11q

Occurrence of any one or more of the above 4 aberrations may result in abnormally high rate of development and proliferation of B-cells in the bone marrow and other hematopoietic tissue. This results in crowding of normally developing cells in the marrow as well as crowding of normally maturing cells in the lymph nodes. As a result, the B-cells develop impaired functioning even though the lymphocytic count keeps on increasing.

Epidemiology

Incidence

CLL is the most common leukemia of adults in the Western world. There are about 15,000 new cases of CLL each year in the United States [1].

Age and sex

The median age at diagnosis is 60 years, and there is a 2:1 male predominance [1].

Race

This disease is very rare in the Chinese and related races [2].

Sex distribution
Age distribution

Pathophysiology

Certain bio-markers may be present in CLL. Most common such markers are B-cell CD19 and B-cell CD20. CD23 and CD5 may also be found.

Morphology

Electron microscopy of blood smears show diffuse effacement of lymph node architecture, with the presence of proliferation centres. Stromal cells in proliferation centres seem to express a variety of factors that stimulate the activity of the transcription factor NK-kB, [6], which promote cell growth and survival. This factor, along with others, result in uncontrolled proliferation of B cells.

The leukemic cells have fragile nuclei, so during slide preparation, insertion of a cover slip on top of the sample on the slide crushes the fragile nuclei, resulting in characteristic 'smudge cells' or 'parachute cells'.

Staging

Binet classification

  • Clinical stage A: This stage is characterised by less than 3 areas of lymphoid enlargement. There is no anemia or thrombocytopenia.
  • Clinical stage B: In this stage, 3 or more than 3 areas of lymphoid enlargement are found. There is no or little evidence of anemia or thrombocytopenia.
  • Clinical stage C: In this most serious stage, there is anemia and/or thrombocytopenia regardless of how many areas of lymphoid enlargement are present.

Rai classification

  • Stage 0: Only lymphocytosis is present i.e >15,000 mm3.
  • Stage I: Lymphocytosis along with lymphadenopathy is present.
  • Stage II: Lymphocytosis along with either splenomegaly or hepatomegaly is present. Lymphadenopathy may or may not be present.
  • Stage III: Absolute lymphocytosis with anemia is present. Hepatosplenomegaly and lymphadenopathy may or may not be present.
  • Stage IV: Absolute lymphocytosis with thrombocytopenia (<100,000/mm3). Lymphadenopathy, hepatosplenomegaly and anemia may or may not be present.

Prevention

There is no known prevention of this disease. However, routine check ups and examinations may help in preventing the disease from reaching an advanced stage.

Summary

Chronic lymphocytic leukemia (chronic lymphoid leukemia, CLL) belongs to the lymphoproliferative disorders and according to the classification of the World Health Organization to the Non-Hodgkin lymphomas and neoplasias of the B lymphocytes. CLL is the most common form of leukemia in the Western world and affects mainly people of advanced age. CLL leads to unspecific symptoms such as general malaise and weakness, fever, night sweats, weight loss and enlarged lymph nodes, spleen or liver. The onset of these symptoms is often insidious and CLL is frequently diagnosed in routine examinations.

Patient Information

CLL is the most common white blood cell cancer in US adults. It is more common in males and occurs most commonly in the late 5th or early 6th decade of life. Common symptoms are enlarged lymph nodes, fever, night sweats, fatigue, weight loss and frequent infections.

References

Article

  1. Robbins and Cotran, Pathological Basis of Disease. 8th ed. Chap 13, 596-638.
  2. Davidson's Principles and Practice of Medicine. 16th ed. Chap 14,737-39.
  3. Harris NL, Jaffe ES, Diebold J, et al. WHO Classification of neoplasticism disease of the hematopoietic and lymphoid tissues:a report of the Clinical Advisory Committee meeting Airlie-house, Virginia, Nov 1997. J Clin Oncol. 17(12):3835-49
  4. Slager SL, Kay NE. Familial chronic lymphocytic leukemia: What does it mean to me? Clin Lymphoma Myeloma. Sep 1,2009;9:S194-S197
  5. Dohner H, Stilgenbauer S, Benner A, et al. Genomic Aberrations and survival in chronic lymphocytic leukemia. NEJM 2000 343(26):1910-6
  6. Endo T, et al. BAFF and APRIL support chronic lymphocytic leukemia B-cell survival through activation of the canonical NF- kappaB pathway. Blood 109:703, 2007
  7. Tsimberiou AM, Keating MJ. Richter's transformation in chronic lymphocytic leukemia. Semin Oncol 33:250, 2006
  8. Alinari L, et al. Alemtuzumab (Campath-1H) in the treatment of chronic lymphocytic Leukemia. Oncogene 26:3644, 2007
  9. Dreger P, Brand R, Hansz J, et al. Chronic leukemia working party of EBMT 2003. Treatment related mortality and graft versus-leukemia activity after allogenic stem cell transplantation for CLL using intensity-reduced conditioning. Leukemia 17(5);841-8

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Last updated: 2018-06-21 20:03