Patients usually present on diagnosis with pancytopenia, splenomegaly, and circulating hairy cells [4]. All the signs and symptoms of hairy cell leukemia are the result of the proliferation of the abnormal white blood cells. All of the symptoms are reversible by reducing the number of malignant cells [5].

Signs and symptoms include the following [5] [7]:

• Splenomegaly 60–70%
• Hepatomegaly 40–50%
• Abdominal lymph node enlargement 10%
• Weight loss
• Anemia 70%
• Thrombocytopenia 80%
• Neutropenia 75%
• Monocytopenia 90%
• Lymphadenopathy
• Hairy cells in peripheral blood 95%
• Adult respiratory distress syndrome 
• Immunosuppression
• Decreased functional B-cells
• Increased numbers of abnormal white blood cells
• Hypercoagulation or intravascular coagulation
• Hyperkalemia
• Hyperkalemia

The cause of hairy cell leukemia is not known, however exposure to toxic chemicals, radiation, and wood dust have been implicated. The mechanism involved in the development of the abnormal hairy leukocytes is as yet unknown.

• Splenomegaly

  Also these cytokines may play a role in the development of hairy cell leukemia.Key words: hairy cell leukemia -  sarcoidosis - massive splenomegaly. [ncbi.nlm.nih.gov]

• Massive Splenomegaly

  Also these cytokines may play a role in the development of hairy cell leukemia.Key words: hairy cell leukemia -  sarcoidosis - massive splenomegaly. [ncbi.nlm.nih.gov]

  Definition / general Rare chronic leukemia that commonly presents with massive splenomegaly, with tumor cells containing hair-like projections Terminology Formerly known as leukemic reticuloendotheliosis Clinical features Mean age 52 years, range 20 - [pathologyoutlines.com]

  The decision to treat is based on symptomatic cytopenias (low blood counts), massive splenomegaly (enlarged spleen), or the presence of other complications. [lymphomation.org]

• Abdominal Lymphadenopathy

  Rarely there is marked abdominal lymphadenopathy due to infiltration by large hairy cells. Recurrent opportunistic infections, vasculitis and immune dysfunction are common. [e-immunohistochemistry.info]

  Generalized abdominal lymphadenopathy is not a routine feature of HCL. [hindawi.com]

  Incidence and significance of abdominal lymphadenopathy in hairy cell leukaemia. Leukemia & Lymphoma 1994, 14 suppl 1:79-83. 195. [medical-diss.com]

  Mercieca, 3., Puge, M., Matotes, B., et at Incidence and significance of abdominal lymphadenopathy in hairy-cell leukaemia (abstract). Blood, 1993;82.2273. 16. Seshadri, R. Brown, E. and Zipursky, A. Leuksemic reticuloendotheliosia. [jpma.org.pk]

  The frequency and significance of intra-abdominal lymphadenopathy have not been extensively defined, although one report suggested that lymphadenopathy correlated with overall survival. 8 Multiple investigators have found that age and hemoglobin level [bloodjournal.org]

• Generalized Lymphadenopathy

  Hepatomegaly was seen in 28% cases; less than that reported in other studies. [11],[12] Though uncommon, lymphadenopathy was seen in six cases, two patients had abdominal nodes detected on CT scan, while one patient had generalized lymphadenopathy, and [ijpmonline.org]

• Easy Bruising

  A shortage of blood platelets may lead to easy bruising or bleeding. Swollen lymph nodes: In some cases, the signs of leukemia may include noticeable swelling of the neck, armpit or groin. This occurs when leukemia has spread to the lymph nodes. [cancercenter.com]

  When symptoms appear, they may include one or several of the following: frequent infections fever shortness of breath fatigue or weakness bone pain, particularly below the ribs easy bruises easy bleeding enlarged liver or spleen excessive sweating, particularly [medicalnewstoday.com]

  These include weight loss, fatigue, easy bruising, anemia and recurrent infections. Treatment of hairy cell leukemia involves chemotherapy, immunotherapy, surgery and targeted therapy. Accurate diagnosis is critical for effective treatment. [medindia.net]

• Anemia

  Hairy cell leukemia Chronic leukemia Aplastic anemia Cladribine Pentostatin © AlphaMed Press [doi.org]

  Some HCL patients may receive an initial diagnosis of aplastic anemia and may show a good response to ATG masking the underlying HCL. [ncbi.nlm.nih.gov]

  Blood-work is obtained, showing leukopenia, along with anemia and thrombocytopenia. Physical exam reveals marked splenomegaly. A peripheral blood smear shows cells with hairy cytoplasmic projections. It stains with TRAP. [step2.medbullets.com]

• Fatigue

  He has been feeling very fatigued lately, but attributed that to his multiple infections. Blood-work is obtained, showing leukopenia, along with anemia and thrombocytopenia. Physical exam reveals marked splenomegaly. [step2.medbullets.com]

  During interferon-α therapy she developed multiple subcutaneous swellings, accompanied by fever and fatigue. A skin biopsy revealed lobular, T-cell lymphocytic panniculitis. [ncbi.nlm.nih.gov]

  Some symptoms, like night sweats, fever, fatigue and achiness, resemble flu-like symptoms. [cancercenter.com]

• Lymphadenopathy

  A 47-year-old woman with pancytopenia, excessive systemic lymphadenopathy and splenomegaly was referred to our hospital. [ncbi.nlm.nih.gov]

  […] was seen in 19%, and significant lymphadenopathy was seen in 10% (usually localized). [ijpmonline.org]

  Clinical Information A neoplastic disease of the lymphoreticular cells which is considered to be a rare type of chronic leukemia; it is characterized by an insidious onset, splenomegaly, anemia, granulocytopenia, thrombocytopenia, little or no lymphadenopathy [icd9data.com]

• Weight Loss

  Twenty-five years later, the patient presented with weight loss, fatigue, and a large PET-avid mass surrounding the head of the pancreas. [ncbi.nlm.nih.gov]

  loss for no known reason The causes of hairy cell leukemia are not fully understood. [medicalnewstoday.com]

• Recurrent Infection

  Patients require treatment when they have significant cytopenia or recurrent infections. [ncbi.nlm.nih.gov]

  The clinical features are often related to cytopenias and include recurrent infections (due to neutropenia) and weakness and fatigue (due to anemia). Monocytopenia is one of most sensitive markers of HCL. [webpathology.com]

  Common hairy cell leukemia symptoms Symptoms of HCL may include: Weakness or feeling tired Recurrent infections and fevers Easy bruising or bleeding Shortness of breath Unexplained weight loss Pain or a feeling of fullness below the ribs Painless lumps [cancercenter.com]

  Treatment may be advised for HCL patients with low blood cell counts, recurrent infections, or an enlarged spleen or lymph nodes. Treatment is most often with chemo using one of the purine analog drugs -- either cladribine (2-CdA) or pentostatin. [cancer.org]

• Abdominal Pain

  Most symptomatic patients present with complaints of fatigue, bruising, and abdominal pain. Other patients are seen initially for infections due to cytopenia. [symptoma.com]

  They may also have left upper quadrant (LUQ) abdominal pain, frequent infections, bruising, and weight loss. Physical examination may reveal splenomegaly, with or without hepatomegaly and lymphadenopathy. [hematologyandoncology.net]

  Other rare complications include splenic rupture, which although rare, may cause severe abdominal pain and symptoms of hypotensive shock. [cancertherapyadvisor.com]

  In addition, affected individuals may experience abdominal pain and a feeling of abdominal fullness due to an abnormally enlarged spleen (splenomegaly). [rarediseases.org]

• Hepatomegaly

  No hepatomegaly or lymphadenopathy was detected. [ncbi.nlm.nih.gov]

  Physical examination revealed splenomegaly in most patients (92%) and hepatomegaly in a minority (28%). [ijpmonline.org]

  Hepatomegaly and liver dysfunction may be found in 20% of patients. Approximately 30% of patients diagnosed with hairy cell leukemia develop secondary cancers during or following treatment for the disease. [symptoma.com]

  […] with filamentous, hair-like projections tartrate-resistant acid phosphatase (TRAP +) Presentation Symptoms pancytopenia from infiltration of bone marrow fatigue infections bleeding anemia Physical exam splenomegaly from infiltration of spleen may see hepatomegaly [step2.medbullets.com]

  The most frequent initial manifestations are abdominal discomfort or distension, usually related to splenomegaly, hepatomegaly and manifestations derived from cytopenias such as anemia, bleeding and/or infections. [orpha.net]

• Hepatosplenomegaly

  Physical examination revealed no hepatosplenomegaly or lymphadenopathy, and peripheral blood analysis demonstrated a moderate thrombocytopenia, mild neutropenia, mild normocytic anemia, and normal biochemical analysis. [ashpublications.org]

  Abdominal discomfort is a common symptom, resulting from hepatosplenomegaly. Splenomegaly is present in virtually all cases, and is classed as massive in more than 80% of cases. [orpha.net]

  The major clinical manifestations of malaise, hepatosplenomegaly, anemia, and leukocytosis are related to abnormal, excessive, unrestrained overgrowth of granulocytes in the bone marrow. [medical-dictionary.thefreedictionary.com]

  PR required all of the following: (1) less than a CR, (2) circulating HCs less than 5% and reduction of more than 50% of HCs in the bone marrow, (3) normalization of peripheral blood counts, and (4) more than 50% reduction of abnormal adenopathy or hepatosplenomegaly [doi.org]

• Purpura

  Autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia purpura (ITP) can also be observed. 7 Indicators to initiate therapy include fatigue and weakness that interfere with quality of life, symptomatic organomegaly, frequent infections, and signs [hematologyandoncology.net]

  Severe immune thrombocytopenic purpura and hemolytic anaemia in a hairy-cell leukaemia patient. Eur J Haematol 1995; 54 : 127–129. 7. Mainwaring CJ, Walewska R, Snowden J, Winfield DA, Ng JP, Chan-Lam D et al. [nature.com]

  Hemorrhagic manifestations, such as purpura and echymoses, caused by severe thrombocytopenia with peripheral blood plasma platelet counts of less than 80,000/mm3, are found in about 20% of patients(2). [scielo.br]

  […] tehniliste tõkete kõrvaldamist käsitlevate direktiivide tehnika arengule kohandamise komitee arvamusega en Low red blood cells, low blood platelets Kidney damage with low blood platelets and low red blood cell counts with or without rash (thrombocytopenic purpura [et.glosbe.com]

  Splenectomies are also performed for patients whose persistently enlarged spleens cause significant discomfort or in patients whose persistently low platelet counts suggest idiopathic thrombocytopenic purpura. [en.wikipedia.org]

• Cutaneous Manifestation

  However, data regarding the treatment of these cutaneous manifestations are lacking, given the rarity of the concomitance of HCL and vasculitic syndromes. [ncbi.nlm.nih.gov]

  In addition, patients may present with cutaneous manifestations, such as herpes zoster, cellulitis, abscess, pyoderma, dermatophytosis, leukemia cutis, ecchymosis, and purpura. 7 Laboratory findings may reveal leukopenia, neutropenia, monocytopenia, anemia [hematologyandoncology.net]

• Petechiae

  […] symptoms generally last longer than two weeks, and may include: Fever, chills Fatigue, weakness Loss of appetite, weight loss Night sweats Bone/joint pain Abdominal discomfort Headaches Shortness of breath Frequent infections Easy bruising or bleeding Petechiae [cancercenter.com]

Patients may be asymptomatic and the disease is only identified when a complete blood count is done for an unrelated complaint [7]. Most symptomatic patients present with complaints of fatigue, bruising, and abdominal pain. Other patients are seen initially for infections due to cytopenia [7].

Hairy cell leukemia is suspected initially when the abnormal hairy cells are present in the peripheral blood. Diagnosis is confirmed by demonstration of hairy cells in biopsy specimens from the bone marrow or spleen or in peripheral blood [9]. Bone marrow aspiration and the presence of the abnormal cells is the least invasive means of making a definitive diagnosis [1]. However, bone marrow aspiration is often impossible because of fibrosis of the marrow. Bone marrow biopsy is then the method of choice and the final step in confirming the diagnosis [1].

Diagnostic tests include [7]:

• Peripheral blood smear
• Bone marrow biopsy
• Immunohistology to exclude other B-cell leukemia
• Monoclonal antibody panel
• Computed tomography of splenomegaly and hepatomegaly

The most consistent laboratory finding is cytopenia, usually affecting all three types of blood cells: red cells, white cells, and platelets [7]. Physical examination may show significant signs of anemia, pale skin and mucosa, elevated pulse and respiratory rate, fatigue and exercise intolerance. In most cases splenomegaly is a presenting sign.

• Neutropenia

  Toxicities—Duration of neutropenia, febrile episodes, and hospitalization. [bloodjournal.hematologylibrary.org]

  Polycythemia vera is a myeloproliferative disease that sometimes evolves to myelofibrosis, causing splenomegaly and neutropenia. [ncbi.nlm.nih.gov]

  This can be neutropenia, anaemia or thrombocytopenia [3] Neutropenia Not enough neutrophils in the blood. [cllsupport.org.uk]

• Monocytopenia

  In fact, in 90% of patients the abnormal blood counts, including the monocytopenia [8], are corrected within 3 to 6 months with subcutaneous injections three times a week. [cllsupport.org.uk]

  We present a case of a 52-year-old man hospitalized in July 2012 in the Clinic of Hematology of Craiova, Romania, having splenomegaly, leukocytosis with lymphocytosis, anemia and thrombocytopenia, without monocytopenia, which exposed, in the peripheral [ncbi.nlm.nih.gov]

  Intriguingly, our patient was diagnosed with HCL following a random hematological evaluation (required to start with isotretinoin therapy) and did not show typical characteristics of HCL, such as hepatosplenomegaly, pancytopenia, and monocytopenia, suggesting [ashpublications.org]

• Lymphocytosis

  We report the simultaneous presence of hairy cell leukemia, monoclonal B-cell lymphocytosis, and alpha beta CD4(++) /CD8(+) T-cell large granular lymphocytosis in a 63-year-old man. [ncbi.nlm.nih.gov]

  HCL-v makes up 10% of all these cases and presents in older patients with more severe disease and higher lymphocytosis. [karger.com]

• Leukopenia

  Blood-work is obtained, showing leukopenia, along with anemia and thrombocytopenia. Physical exam reveals marked splenomegaly. A peripheral blood smear shows cells with hairy cytoplasmic projections. It stains with TRAP. [step2.medbullets.com]

  Later he developed leukopenia and pancytopenia. Bone marrow biopsies showed fibrosis. Further morphological analyses of bone marrow and blood smears revealed probable transformation into acute myeloid leukemia. [ncbi.nlm.nih.gov]

  Case Report In August 2012, a 65-year-old white woman presented for evaluation of a 14-month history of leukopenia, neutropenia, and monocytopenia, which were initially noticed during a routine complete blood count (CBC) that revealed the following: white [hematologyandoncology.net]

  Radiotherapy of the spleen of one patient resulted in a prompt leukopenia and a delayed decrease in the size of the spleen. This patient is alive and asymptomatic 21 months after diagnosis. [bloodjournal.hematologylibrary.org]

• Monocytosis

  The differential diagnosis of leukemia includes other neoplastic hematopoietic disorders, such as lymphoma, myelodysplastic syndromes, multiple myeloma, aplastic anemia, severe megaloblastic anemia, severe lymphocytosis, severe monocytosis, and bone marrow [ncbi.nlm.nih.gov]

  Symptoms include splenomegaly, monocytosis with granulocytosis, and thrombocytopenia. leukemia cu´tis leukemia with leukocytic invasion of the skin marked by pink, reddish brown, or purple macules, papules, and tumors. eosinophilic leukemia a form of [medical-dictionary.thefreedictionary.com]

• Leukocytosis

  The HCL patients had a significantly higher frequency of leukopenia (p = 0.024) and monocytopenia (p = 0.008) and a lower frequency of leukocytosis (p = 0.018) than HCL-v patients. [ncbi.nlm.nih.gov]

  (C) EFS in HCL with leukocytosis versus HCL without leukocytosis. [doi.org]

  Conclusions: Though pancytopenia is common, occasional patient can present with normal blood counts or leukocytosis. [ijpmonline.org]

The goal of therapy in hairy cell leukemia is to decrease the risk of infection and other serious complications [10]. Hairy cell leukemia is diagnosed based on the presence of the characteristic malignant white blood cells called hairy cells in the bone marrow. It is important to exclude other leukemia which present the same but require different treatment.

Once the diagnosis has been made the first determination to be made is if treatment is required at the time. Since all treatment modalities are associated with some side effects deferring treatment until the patient is symptomatic seems appropriate [6] [7]. If the patient has significant cytopenia and its related problems: anemia, infections or bleeding, and/or significant splenomegaly, treatment should be started immediately [7]. 

Currently the initial treatment of choice are Purine analogues (nucleosides) and/or Interferon A. Both are effective treatment regimes, but nucleosides are the first line of treatment because they have a higher complete remission rate [6] [9]. However, there are still instances where Interferon therapy is indicated [9] [11] [12].

Splenectomy was the recommended treatment for hairy cell leukemia until the mid-1980s. The efficacy of splenectomy on survival of patients has not been demonstrated [10]. In the past three decades, three very effective therapies have been approved for use with this disease: Interferon Alfa, pentostatin, and cladribine [4]. Splenectomy is rarely performed currently except in cases of profound splenomegaly or excessive blood burden [4] [11] [12].

Pentostatin and cladribine are the two most commonly used Purine analogues [7] [9] [11] [12]. Studies have shown that a single courses of cladribine orpentostatin administered to patients with hairy cell leukemia have high response rates with a majority of patients achieving complete responses. Most patients then go on to long-lasting complete remissions. These rates have been recorded as high as 90-95%. Patients, approximately 30%, who relapse, may be re-treated successfully with repeat treatment [4] [9] [11] [12].

Alpha-interferon has a complete remission rate of 86 % [9]. Interferon Alfa alone or in conjunction with one of the Purine analogues is indicated in patients with infections at diagnosis. Studies indicate that its inclusion in the treatment regime may increase the survival rate patients with advanced diesease. How this works is unclear [10].

Growth factors such as Granulocyte colony-stimulating factor (G-CSF) may be used in hairy cell leukemia to treat severe neutropenia. It can be given before, during and/or after treatment with a Purine analogue. Its routine use is not recommended [7].

Long-term follow-up with repeat bone marrow biopsy is necessary in order to detect relapse or residual disease early [1]. Relapse has been reported in about 30% of patients. These cases respond well to retreatment with the drugs used in initial therapy (92%) [8].

Follow-up studies have shown a complete remission rate of approximately 95% and a partial response rate of 5%. These rates were often achieved after a single course of treatment [4]. About 37% of treated patients experienced relapse after their first course of treatment. The time to relapse had a median of 42 months [4].

There is no clear guidelines to predict the progress of the disease. Since hairy cell leukemia primarily affects bone marrow and spleen and progresses slowly, prognosis depends on status of both these organs [7].

The response to purine analogue therapy is also a predictor of patient outcome. Those achieving complete remission did better than those with only partial response.  For this reasons, the goal of treatment is achieve a complete remission [7].

The complications of hairy cell leukemia are primarily responsible for the morbidity and mortality associated with the disease. These complications include:

• Recurrent and possibly life threatening infections
• Anemia and subsequent cardiovascular and respiratory disorders
• Problems with coagulation, bleeding
• Splenic rupture
• Secondary malignancies

Complications associated with hairy cell leukemia are due to interruption in the production of normal blood cells. Anemia occurs with low red cell production. Decreased white cell production increases the risk of infection. Low platelet counts result in bleeding or excessive bruising. Abdominal pain and potential splenic rupture are complications resulting from splenomegaly which is present in almost all cases. Hepatomegaly and liver dysfunction may be found in 20% of patients [1] [2].

Approximately 30% of patients diagnosed with hairy cell leukemia develop secondary cancers during or following treatment for the disease [4].

The symptoms seen in hairy cell leukemia are due to the proliferation of malignant B cell leukocytes that infiltrate the cells of bone marrow, resulting in bone marrow failure [1] [4].

All leukemias result from the proliferation immature of blood cell that are not functional. They do not then allow adequate production of normal erythrocytes, lymphocytes, granulocytes, and platelets. This pancytopenia results in severe anemia, life-threatening infection, bleeding, and the possibility of secondary malignancies [5]. Hairy cell leukemia was first described by Bouroncle, et al. in 1958 [9]. Hairy cell leukemia differs from other in two aspects: the presence of the distinctive hairy cells and the fact that it is the most treatable of all the blood malignancies [6].

The progression varies from patient to patient. Some patients may need no treatment; the majority (> 90 %) have complete remissions; some have chronic symptoms for many years; while still others die early from complications [5].

The annual incidence has been estimated to be approximately 1 in 500,000. It is a rare disorder accounting for only 2% of all leukemia cases. It affects men five times more often than women (5:1 incidence) and occurs most commonly in middle-age or older adults (average age of onset is 50 years) [7].

Race

Hairy cell leukemia is seen more often in Caucasians and Ashkenazi Jewish males. It is rare in those of Japanese and African descent [8].

Sex

Hairy cell leukemia occurs most often in males, with a male-female ratio of 5 to 1 [8].

Age

Hairy cell leukemia occurs most often in middle-aged men, with a median age of 52 years [8].

HCL is a rare, highly treatable, chronic B-cell leukemia. They disorder is distinguished by the presences of malignant white blood cells called hairy cells because of their characteristic appearance. These cells also possess a rare acid phosphatase enzyme (tartrate-resistant acid phosphatase (TRAP)) which acts as a useful marker for the disease [1] [6].

The abnormal cells proliferative slowly, so that symptomatology results from an abnormally prolonged cell survival rate [12]. The effect of the accumulation of hairy cells on the other blood cell types is pancytopenia. The etiology of this is unclear [6].

The distinctive biological features include [6]: 

• Monocytopenia
• Distinctive tissue distribution
• Distinctive patterns of tissue remodeling

In hairy cell leukemia all types of blood cells are affected resulting in:

• Erythrocytopenia
• Thrombocytopenia
• White blood cell count decreased
• Neutropenia

Unlike other lymphoid disorders, hairy cell leukemia has distinctive tissue distribution [6]:

• Hairy cells do not infiltrate lymph nodes.
• The spleen is more often involved and the pattern of splenic involvement is distinctive.
• Hairy cells also accumulate in the liver, without hepatomegaly

Hairy cell leukemia also has a distinctive pattern of bone marrow fibrosis. 

Since the cause of hairy cell leukemia is not known, ways to prevent the disease are not clear. Some toxic chemicals and radiation have been implicated in the incidence of the disorder. Avoidance of these environmental substances may prevent the disease. 

Hairy cell leukemia (HLC) is a chronic form of B-lymphocytic leukemia in which the predominant feature is the presence of characteristic malignant white blood cells [1] [2]. These cells are named hairy cells because of protrusions on the surface of the cells that appear hair like [1]. These malignant cells infiltrate the bone marrow, spleen, peripheral blood, and, occasionally, the liver [3]. The primary symptoms are anemia, pancytopenia, and splenomegaly [4].

Hairy cell leukemia is a rare disorder, accounting for only 2% of all cases of leukemia [1] [4]. It is a very treatable form of blood malignancy. Its diagnosis and differentiation from other forms of leukemia is of prime importance so that appropriate treatment is instituted as soon as possible.

The etiology of hairy cell leukemia is not known. But exposure to chemicals and radiation has been implicated. The highest incidence of Hairy cell leukemia is seen in middle-aged men [3]. It is five times as prevalent in men as in women [3] [4]. With chemotherapy complete remission occurs in about 90% of patients. The disease may be mild and slowly progressive so that patients do not need treatment. Complications include recurrent and life threatening infections, severe anemia, bleeding, and ruptured spleen.

What is hairy cell leukemia?

Hairy cell leukemia is a rare, highly treatable form of leukemia, blood cell cancer. Leukemia is a blood disorder where abnormal white blood cells are produced instead of normal cells. The malignant cells in this disease are called hairy cells because of the hair-like protrusions on the surface of the cell. In hairy cell leukemia the abnormal cells also interfere with the production of all blood cell types, red blood cells, white blood cells, and platelets.

What are the symptoms?

The symptoms are the result of the suppression of normal blood cells. They include:

• Fatigue
• Anemia
• Bleeding
• Increased susceptibility to infection
• Greatly enlarged spleen
• Increased heart or respiratory rate
• Liver enlargement and dysfunction

What causes hairy cell leukemia?

The cause of hairy cell leukemia is not known. Exposure to toxic chemicals and radiation have been suspected but not proven to cause the disease.

Who gets hairy cell leukemia?

Anyone can get hairy cell leukemia, however it occurs most often in middle aged or elderly Caucasian men. The mean age at diagnosis is 50 years. The incidence in men is 5 times as high as in women.

How is it diagnosed?

Diagnosis is based on the results of the physical examination, blood tests, bone marrow biopsy and CT scan. The initial suspicion may occur with a routine blood test. Confirmation is based on the presence of characteristic malignant white blood cells called hairy cells in the bone marrow.

How is hairy cell leukemia treated?

Hairy Cell leukemia can be treated with intravenous medications that interfere with the production of the abnormal cells. Complete remission occurs in nearly 90% of patients. Usually only one course of treatment is necessary. However relapse occurs in approximately 30% of patients. Relapse can be treated with another course of treatment with a 90% success rate.

What are the complications?

The complications of hairy cell leukemia are due to the decrease in the production of normal blood cells. They include:

• Recurrent and possibly life-threatening infections
• Cardiac and respiratory failure due to severe anemia
• Hypo-coagulation and bleeding due to decreased platelets
• Ruptured spleen

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