Prolymphocytic leukemia

Prolymphocytic leukemia Main page
Patient Information B-cell prolymphocytic leukemia Patient Information T-cell prolymphocytic leukemia Patient Information
Overview
Causes
Classification B-cell prolymphocytic leukemia B-cell prolymphocytic leukemia
Differentiating B-cell prolymphocytic leukemia from T-cell prolymphocytic leukemia

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Carlos A Lopez, M.D. [2], Maria Fernanda Villarreal, M.D. [3]

For more information regarding B-cell prolymphocytic leukemia, please click here.
For more information regarding T-cell prolymphocytic leukemia, please click here.

Overview

Prolymphocytic leukemia is a rare lymphoid leukemia, which account for only about 2% of all mature lymphoid leukemias. Prolymphocytic leukemias present similar to leukemia. Like lymphomas, they originate in the lymphocytes, but do not form solid tumors. Prolymphocytic leukemias are also considered lymphoproliferative disorders, which lymphocytes are produced in large amounts. Prolymphocytic leukemia may be classified according to the type of cell involved: B-cell prolymphocytic leukemia and T-cell prolymphocytic leukemia. Originally, it was thought to be a rare variation of chronic lymphocytic leukemia, but is now considered a distinct disease. It is usually classified as a kind of chronic lymphocytic leukemia. Although these 2 types of prolymphocytic leukemias share some of the same characteristics, the World Health Organization (WHO) classifies them as different types of lymphoid leukemias.

Classification

For more details about each specific type of prolymphocytic leukemia, please select:

Prolymphocytic leukemia

B-cell prolymphocytic leukemia

T-cell prolymphocytic leukemia

Differentiating B-Prolymphocytic Leukemia from T-Prolymphocytic Leukemia

In order to distinguish B-prolymphocytic leukemia from T-prolymphocytic leukemia, see the table below.

Characteristics	B-cell Prolymphocytic Leukemia	T-cell Prolymphocytic Leukemia
Epidemiology	Rare	Very rare
Age	60-70 years	60-70 years
Onset	Elevated white blood cell count > 100,000/microL Hepatosplenomegaly	Elevated white blood cell count > 100,000/microL Splenomegaly Generalized lymphadenopathy Occasional skin involvement
Clinical Features	B symptoms Fever Swelling of lymph nodes Night sweats Persistent fatigue	Fever Persistent fatigue Weight loss
Diagnosis	Peripheral blood smear Prolymphocytes (90%) Biomarkers CD20+	Peripheral blood smear Medium-sized lymphoid cells Moderately condensed chromatin and a visible nucleolus Biomarkers CD52+

Differentiating Prolymphocytic Leukemia from other Leukemias

Differential Diagnosis	Surface Immunoglobulin	CD5	CD22/FMC7	CD23	CD79b	CD103
Prolymphocytic leukaemia	Strongly positive	Negative	Positive	Negative	Positive	Negative
Hairy cell leukaemia	Strongly positive	Negative	Positive	Negative	Positive/Negative	Positive
Chronic lymphocytic leukaemia	Weakly positive	Positive	Negative	Positive	Negative	Positive/Negative
Mantle cell lymphoma	Positive	Positive	Strongly positive	Negative	Strongly positive	Negative
Follicular lymphoma	Strongly positive	Negative	Positive	Negative	Strongly positive	Negative

Disease	Etiology	Clinical Manifestation				Laboratory Findings		Gold standard diagnosis	Associated findings
		Demography	History	Symptoms	Signs	Lab	Histopathology
Acute myelogenous leukemia	Clonal proliferation of malignant myeloid blast cells in the marrow Genetic abnormalities t(8;21), inv(16), and t(15;17)	The most common leukemia in adults Median age of 63 years old	Smoking, previous chemotherapy or radiation therapy, myelodysplastic syndrome, and exposure to the chemical benzene	Fatigue Bleeding	Bone tenderness Dyspnea Leukemia cutis Swelling of the gums Chloroma Rare LAP	Anemia Thrombocytopenia Leukocytosis or leukopenia	Leukemic blasts Positive Auer rods	Flow cytometry > 20% blasts of myeloid lineage	Persistent or frequent infections Fatal within weeks or months if left untreated Down syndrome or Bloom syndrome
Acute lymphoblastic leukemia	Arrest of lymphoblasts Chromosomal translocations: t(9;22) , t(12;21), t(5;14), t(1;19)	The most common cancer in children Peak 2-5 years of age Boys > girls	History of cancer History of drug exposure	Generalized weakness Fatigue Bleeding	Hepatosplenomegaly LAP Dyspnea Pallor Papilledema Nuchal rigidity Cranial nerve palsy Testicular enlargement Mediastinal mass	Anemia Thrombocytopenia Normal or slightly increased WBC counts	Lymphoblasts Atypical cells	Bone marrow biopsy	CNS involvement
Chronic myelogenous leukemia	Dysregulated production and uncontrolled proliferation of mature and maturing granulocytes BCR-ABL1 fusion gene Autosomal dominant mutation	Median age 50 years old		Generalized weakness Fatigue Early satiety Abdominal fullness Bleeding	Asymptomatic Blast crisis Excessive sweating Papilledema Tenderness over the lower sternum Hepatosplenomegaly	Anemia WBC > 100,000/microL Absolute basophilia and eosinophilia Plt > 600,000 to 700,000/microL Low leukocyte alkaline phosphatase High uric acid	All cells of the neutrophilic series, from myeloblasts to mature neutrophils Myelocyte bulge	Bone marrow biopsy	Acute gouty arthritis Venous obstruction
Disease	Etiology	Demography	History	Symptoms	Signs	Lab	Histopathology	Gold standard diagnosis	Associated findings
Chronic lymphocytic leukemia	Progressive accumulation of monoclonal B lymphocytes	The most common leukemia in adults in western countries M > F Median age 70 years old	Positive family history Exposure to herbicides or insecticides	Bleeding Abdominal pain Generalized weakness Anorexia	LAP (Most common sign) Hepatosplenomegaly Skin lesions (leukemia cutis) Night sweats Muscle wasting	Anemia Thrombocytopenia Absolute lymphocytosis >5000 cells/μl Neutropenia Positive direct Coombs test Hypogammaglobulinemia Elevated lactate dehydrogenase and beta-2 microglobulin	Presence of smudge cells Monoclonality of kappa and lambda producing B cells Express CD19, CD20, CD23, and CD5 on the cell surface	Flow cytometry of the peripheral blood	Extranodal involvement of skin, kidney, lung, spine Membranoproliferative glomerulonephritis Autoimmune hemolytic anemia
Hairy cell leukemia	Accumulation of small mature B cell lymphoid cells with abundant cytoplasm and "hairy" projections BRAF mutation	Uncommon Median age 50 to 55 years old M >> F More common in Caucasians than Blacks	Exposures to ionizing radiation, pesticides, and farming	Generalized weakness Fatigue Early satiety Abdominal fullness Bleeding	Asymptomatic Splenomegaly Spontaneous splenic rupture Skin rash Ascites Pleural effusion	Cytopenia Leukocytosis in 10 to 20 percent Azotemia Abnormal liver function tests Hypergammaglobulinemia	Pancytopenia with monocytopenia and circulating tumor cells characteristic of HCL Dry bone marrow	Analysis of peripheral blood + immunophenotyping by flow cytometry	Vasculitis
Large granular lymphocytic leukemia​	Clonal proliferation of cytotoxic T cells Dysregulation of apoptosis through abnormalities in the Fas/Fas ligand pathway	Rare Median age 60 years M = F	Autoimmune diseases Lymphoproliferative disorders	Generalized weakness Fatigue	Mostly asymptomatic	Modest lymphocytosis Neutropenia Anemia Thrombocytopenia	Large lymphocytes with a condensed round or oval nucleus, abundant pale basophilic cytoplasm, and small azurophilic granules	Biopsy and flow cytometry + T-cell receptor gene rearrangement studies	Recurrent bacterial infection
Chronic neutrophilic leukemia	Mature granulocytic proliferation in the blood and marrow Point mutations in the CSF3R gene	Very rare M = F	Multiple myeloma	Generalized weakness Fatigue	Hepatosplenomegaly Pruritus Gout	Peripheral blood neutrophilia (> 25 x 109/L) with myeloid precursors (promyelocytes, myelocytes, metamyelocytes) Elevated leukocyte alkaline phosphatase	Toxic granulation in the neutrophils Nuclear hypersegmentation Increased myeloid:erythroid ratio > 20:1	WHO diagnostic criteria include leukocytosis of ≥ 25 x 109/L More than 80% neutrophils, Less than 10% circulating neutrophil precursors with blasts	Poor prognosis Absence of the Philadelphia chromosome or a BCR/ABL fusion gene
Disease	Etiology	Demography	History	Symptoms	Signs	Lab	Histopathology	Gold standard diagnosis	Associated findings
Chronic eosinophilic leukemia				Constitutional Rash Rhinitis Gastritis Thromboembolismrelated	Hypertension Eczema, mucosalulcers, erythema Angioedema Ataxia Anemia Lymphadenopathy Hepatosplenomegaly	Leukocytosis with left shift Eosinophilia Basophilia Monocytosis Anemia Thrombocytopenia ↑ B12 levels ↑ LDH	Hypercelluar with ↑ eosinophilicprecursors, ↑ eosinophils, and atypical mononuclear cells		Heart failure Lung fibrosis Encephalopathy Erythema annulare centrifugam
Chronic monocytic leukemia
Prolymphocytic leukemia (PLL)
T-cell large granular lymphocytic leukemia (TLGL)
Disease	Etiology	Demography	History	Symptoms	Signs	Lab	Histopathology	Gold standard diagnosis	Associated findings
Aggressive NK-cell leukemia (ANKL)
Adult T-cell leukemia/lymphoma (ATLL)
Sezary syndrome
Myelodysplastic syndrome				Constitutional symptoms Bleeding	Pallor Petechiae Organomegaly	Pancytopenia	Hypercellular/ normocellular bone marrow with dysplastic changes Macro-ovalocytes Basophilic stippling Howell-Jolly body	Biopsy	Leukemia transformation Acquired pseudo-Pelger-Huët anomaly Infection
Myeloproliferative disorders
Leukemoid reaction

References

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