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Dyspnea, pancytopenia,

Sheena Boury, MD;

Jeffrey S. Morgeson, MD
University of Cincinnati/

and splenomegaly
The Christ Hospital Family
Medicine Residency
(Dr. Boury); Residency
Faculty (Dr. Morgeson)

An extensive initial laboratory assessment, including sheenaboury@hotmail.

testing for viral disorders, left the diagnosis unsolved.
The authors reported no
potential conflict of interest
relevant to this article.

CASE c A 47-year-old man with a history of al- count was 346/mcL. Hemoglobin was 5.4 g/dL
coholism came to our emergency department and platelet count was 47,000/mcL. (Pancy-
(ED) with a 3-week history of sore throat and topenia is defined as hemoglobin <13.5 g/dL
dry cough. He said that for the past 2 months [males] or 11.6 g/dL [females], platelet count
he had experienced worsening shortness of <150,000/mcL, and WBC count <4000/mcL.
breath, increasing weakness, and episodes of Criteria for severe pancytopenia include an
light-headedness. He also said that his gums ANC <500/mcL, platelet count <20,000 mcL,
occasionally bled when he brushed his teeth. and corrected reticulocyte count <1%.1,2)
Our patient owned a farm where he was A repeat complete blood count (CBC)
exposed to pesticides and fertilizers, but he showed similar results. Basic metabolic panel,
reported no contact with sick individuals, new chest x-ray film, and electrocardiogram results
medications, or recent travel. The patient had were all normal.
a 40 pack-year smoking history, but he had Based on the initial lab work, we ordered
quit within the past year. His family history further testing for human immunodeficiency
was negative for malignancies or rheumato- virus (HIV), Epstein-Barr virus (EBV), cytomeg-
logic diseases. alovirus, hepatitis, parvovirus B19, and anti­
On physical exam, we noted splenomeg- nuclear antibodies. All results were negative.
aly (spleen was approximately 3 cm below B12 and folate levels were low normal, and the
costal margin); all other exam findings were reticulocyte count was 2.31%. We admitted
within normal limits. the patient for evaluation of his pancytopenia.
Lab results revealed pancytopenia: the
patient’s serum white blood cell (WBC) was ● WHAT ADDITIONAL TESTING
900/mcL, the absolute neutrophil count (ANC) WOULD YOU PURSUE AT THIS
was 447/mcL, and the absolute lymphocyte POINT?

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in the left-hand navigation bar Vol 60, No 10 | OCTOBER 2011 | The Journal of Family Practice 593
We ordered a bone marrow biopsy and pe- bruising and bleeding secondary to severe
ripheral smear, which is protocol in a case pancytopenia are also seen in about 25% of
such as this. Results showed leukemic and cases.5,6 However, 25% of patients are ini-
“hairy” cells. tially asymptomatic and are found to have
abnormal lab values during a routine well
Hairy cell leukemia
Hairy cell leukemia is an uncommon chronic
B-cell lymphoproliferative disorder.3 It repre- Narrowing in
sents 2% of all adult leukemias; the median on a diagnosis
age of onset is 52 years, with a male predomi- The etiology of pancytopenia is broad, but
nance of 4:1. It is 3 times more common in the diagnostic possibilities (TABLE ) narrow
Caucasians than in African Americans. There depending on the pathogenesis of a patient’s
is still some controversy regarding its cell condition: decreased bone marrow produc-
line, and its pathogenesis is still unknown. tion, increased destruction or sequestration
Exposures to ionizing radiation, EBV, organic of cells, inherited/congenital, or idiopathic.
chemicals, woodworking, and farming have z Testing. After an initial CBC, a second
been cited as possible causes.4 CBC should be obtained to confirm the pan-
z Symptoms of hairy cell leukemia cytopenia before ordering further tests.1,7
include fatigue, weakness, and weight loss. Such testing includes a peripheral blood
Abdominal fullness or discomfort due to smear, a reticulocyte count, serum iron stud-
splenomegaly is seen in 25% of cases.5,6 Easy ies, and viral studies such as HIV, EBV, or par-


Is it pancytopenia? The differential3

Increased destruction/sequestration
Decreased bone marrow production of cells Inherited/congenital

Chemotherapy, radiotherapy Liver disease Gaucher disease

Megaloblastic anemia Portal hypertension Fanconi’s anemia
Myelodysplastic syndromes Hypersplenism due to myelo-
or lymphoproliferative disorders
Evan’s syndrome
Aplastic anemia
Infection (eg, brucellosis, leishmaniasis)
Paroxysmal nocturnal hemoglobinuria
Heavy metal poisoning
Lymphoma with bone marrow
Plasma cell myeloma
Infection (eg, parvovirus B19, CMV)
Anorexia nervosa


Connective tissue disorders (eg, SLE, RA)


Mycobacterial infection; sepsis; acute viral infection (eg, CMV, HIV, EBV)
AIDS, acquired immune deficiency syndrome; CMV, cytomegalovirus; EBV, Epstein-Barr virus; HIV, human immunodeficiency virus;
RA, rheumatoid arthritis; SLE, systemic lupus erythematosus.

594 The Jour nal of Family Pr ac tice | OC TOB ER 2 0 1 1 | V o l 6 0 , N o 1 0

dyspnea, pancytopenia, splenomegaly

vovirus, along with an autoimmune profile interval of 40 months after initial diagnosis.10
and liver function tests.8 A hematology con- This increased risk may be related to im-
sult with probable bone marrow aspirate is munosuppression due to hairy cell leukemia
also indicated.8 or its treatment. The incidence of a second
Diagnosis is made based on clinical find- malignancy occurring before the diagnosis of
ings and lab work revealing pancytopenia. A hairy cell leukemia is 10.2%, and concurrent-
peripheral smear exhibiting leukemic cells ly with the diagnosis is 2.6%.10 This finding
and “hairy” cells suffices to make the diag- suggests some pretreatment predisposition
nosis, although bone marrow biopsy—which to cancer; further studies are being carried
reveals tartrate-resistant acid phosphatase– out to evaluate this matter. The most com-
positive cells on cytochemical staining in mon solid tumors include prostate cancer,
95% of cases3—is often use to corroborate skin cancer, lung cancer, and gastrointestinal
the diagnosis. More recently, immunotyping adenocarcinomas.10
using flow cytometry has become the stan-
dard for confirming the diagnosis of hairy cell Our patient’s outcome
leukemia.3 During our patient’s hospital admission, he
received 6 units of packed red blood cells,
3 daily injections of 480 mcg subcutaneous
Adjust treatment to symptoms filgrastim for 5 days, and ongoing vitamin
and severity of pancytopenia B12 supplementation to assist myeloid re- Hairy cell
Indications for treatment include the devel- covery. Upon discharge, his ANC level had leukemia’s
opment of one or more the following: ANC increased to 1634/mcL. He began outpa- pathogenesis
<1000/mcL with repeat infections; symp- tient treatment with cladribine under the is still unknown,
tomatic anemia with hemoglobin <11 g/dL; care of a hematologist. After almost a year but exposures
platelet count <100,000/mcL associated with of treatment, our patient is in complete to ionizing
bleeding, symptomatic splenomegaly, or ad- remission. JFP radiation,
enopathy; or constitutional symptoms.9 Epstein-Barr virus,
First-line therapy is with one of the cyto- Correspondence
Sheena Boury, MD, Christ Hospital Family Medicine, 830
toxic agents, either cladribine or pentostatin. Thomas More Parkway, Edgewood, KY 41017; sheenaboury@ chemicals,
Splenectomy is another effective option, with
results that last up to 10 years in 50% of cases.9 and farming have
Another option is interferon-alpha.10 been cited as
Before the advent of cytotoxic agents, the Practice pointers
possible causes.
4-year survival rate for hairy cell leukemia  c Obtain a thorough history to aid in nar-
was reported as 68%. Today, durable remis- rowing the list of potential causes in pancy-
sion is attained for many patients. Even after topenia’s broad differential.
relapse, retreatment yields good responses.
The 5-year survival rate now is higher than  c Arrange for a peripheral smear and bone
85%.11 marrow biopsy to confirm the cause of
Close follow-up
c Screen any patient with hairy cell leuke-
of these patients is key
mia for a second primary malignancy.
Potential complications of hairy cell leuke-
mia include infections, bleeding, anemia,  c Base a treatment decision on the pres-
splenic rupture, and a second primary malig- ence of symptoms and the severity of the
nancy. There is a 2- to 3-fold increased risk of pancytopenia.
developing a second malignancy at a median

1. Ishtiaq O, Baqai HZ, Anwer F, et al. Patterns of pancytopenia approach. J Ayub Med Coll Abbottabad. 2004;16:8-13.
patients in a general medical ward and a proposed diagnostic 2. Wang ES, Berliner N. Hematopoiesis and hematopoietic Vol 60, No 10 | OCTOBER 2011 | The Journal of Family Practice 595
Online at
failure. In: Andreoli TE, Carpenter CCJ, Griggs RC, eds. Cecil
Essentials of Medicine. 6th ed. Philadelphia, Pa: WB Saunders;
3. Lembersky BC, Golomb HM. Hairy cell leukemia: clinical
features and therapeutic advances. Cancer Metastasis Rev.

If you’re not visiting us at, 4. Oleske D, Golomb HM, Farber MD, et al. A case-control in-
quiry into the etiology of hairy cell leukemia. Am J Epidemiol.
here’s what you’re missing
5. Catovsky D. Hairy-cell leukaemia and prolymphocytic leukae-
mia. Clin Haematol. 1977;6:245-268.

This month 6. Golomb HM, Catovsky D, Golde DW. Hairy cell leukemia: a clini-
cal review based on 71 cases. Ann Intern Med. 1978;89:677-683.
7. Kar M, Ghosh A. Pancytopenia. J Indian Acad Clin Med.
Dementia care tips: 2002;3:29-34.
8. Evaluation of pancytopenia. Epocrates online. Available at:
• The coin counting test
• Products that prolong independence Accessed December 8, 2010.
Jill Grimes, MD, family physician, Austin, Texas 9. Grever MR. How I treat hairy cell leukemia. Blood. 2010;115:
10. Au WY, Klasa RJ, Gallagher R, et al. Second malignancies in pa-
tients with hairy cell leukemia in British Columbia: a 20-year
experience. Blood. 1998;92:1160-1164.
4 ways to listen to this audiocast: 11. Zakarija A, Peterson LC, Tallman RS. Hairy cell leukemia. In:
1. Go to Hoffman R, Furie B, Benz EJ, et al, eds. Hematology: Basic
Principals and Practice. 5th ed. Philadelphia, Pa: Churchill
2. Visit

Livingstone; 2009. Available at:

3. Scan this QR code to listen to the audiocast 8&isbn=978-0-443-06715-0&type=bookPage&from=content&
on your smart phone uniqId=279464872-2. Accessed December 8, 2010.
4. Text “SAFE” to 25827 from your
mobile phone and follow the prompt.

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