MT417 - CLINICAL HEMATOLOGY II

Linda Sykora, MT(ASCP)SH

Rotation II Morphology Unit

WBC Cases

Morphology Unit – WBC Cases

For these cases, it is assumed that you have taken advantage of the Rotation I Quiz Reviews to recall the information you mastered during Rotation I. Remember to make use of the Rotation I WBC worksheet, quizzes and Exam.

Before proceeding, you will need to have your Rotation II Morphology Unit objectives and handouts available.

Morphology Unit – WBC Cases

The WBC cases will cover much (but not all) of the material you are responsible for. Use your objectives as a guide to identify material that is new for Rotation II.

For new information, you will be referred to pages in the SL manual, handouts in the Rotation II Morphology unit (pink or lavender color) and/or your McKenzie text.

Note: Throughout the case slides, click on if you would like to view the previous slide.

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For the WBC disorders, your first steps should include evaluation of the WBC count and differential (% and absolute #s) to determine the predominant cell type. The presence or absence of anemia or thrombocyto-penia should also be noted.

Patient age and morphologic changes (i.e., toxic neutrophils or reactive lymphs) can provide clues to a reactive versus a malignant cause.

Most benign disorders can be distinguished from malignant disorders without doing a bone marrow; a marrow exam is generally required to establish a definitive diagnosis of malignancy.

Refer to WBC classification sheet on SL pg 183. P

Which of the following conditions indicate the need fora bone marrow evaluation? a. Immature/blast cells are seen on blood smear. b. Significant neutropenia is present. c. Unexplained pancytopenia exists. d. Cells that exhibit features of abnormal growth, i.e.,dyspoiesis, are seen. e. All of the above.

Refer to Morph unit Bone Marrow Handout (pink)

e. e. All of the above. The bone marrow handoutAll of the above. The bone marrow handout reviews indications for a bone marrow examinationreviews indications for a bone marrow examination and includes a normal bone marrow report.and includes a normal bone marrow report.

It allIt all starts with cell morphologystarts with cell morphology…..the MT/CLS has to…..the MT/CLS has todecide that cells look ‘funny’ and need to be decide that cells look ‘funny’ and need to be

evaluated.evaluated. P

The cells shown may be seen in normal bone marrow.1. What are the cells (A) that make the bone and resemble

plasma cells but are much bigger? 2. What is the multi-lobed cell (B) that produces platelets?3. What is the multi-nucleated cell (C) that removes bone

and resembles cell (B)?See normal BM cells on Morph unit Terms Handout (lavender).

1.1. Osteoblasts; often seen in a group; normal kidsOsteoblasts; often seen in a group; normal kids2.2. MegakaryocyteMegakaryocyte3.3. Osteoclast; has a cookie cutter nucleusOsteoclast; has a cookie cutter nucleus P

A CB

The cell (A) is a normal macrophage/histiocyte that ispresent in the bone marrow, spleen, liver and other tissues.1. The cell (B) is an abnormal macrophage full of the

unmetabolized lipid, glucocerebroside. It is a:2. The cell (C) is an abnormal macrophage full of the

unmetabolized lipid, sphingomyelin. It is a:See Lipid Disorders on Morph unit Terms Handout (lavender).

1.1. Gaucher cell (B) seen in Gaucher’s disease; wrinkled, Gaucher cell (B) seen in Gaucher’s disease; wrinkled, tissue-paper appearance of macrophage cytoplasm.tissue-paper appearance of macrophage cytoplasm.

2.2. Niemann-Pick cell (C) seen in Niemann-Pick disease; Niemann-Pick cell (C) seen in Niemann-Pick disease; foamy/bubbly appearance of macrophage cytoplasm.foamy/bubbly appearance of macrophage cytoplasm.

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A B C

Case AA 4 year old girl is brought to the ER due to fever. Lab data:

WBC 15.2 K/uL; Hgb 12.0 g/dL; PLT 200 K/uL; Differential results:58 segs-25 bands-10 lymphs-7 monosThe appearance of her WBCs are shown.

1. Which of the following best describes her results?a. regenerative left shiftb. degenerative left shiftc. neutrophilic leukemoid reactiond. leukoerythroblastic reaction

1.1. a.a. regenerative left shift; elevated WBC due toregenerative left shift; elevated WBC due to neutrophilia and increased band neutrophilsneutrophilia and increased band neutrophils

(the marrow released immature neutrophils).(the marrow released immature neutrophils). P

Case A2. How did you rule out the other possibilities (b,c,d)? 3. Her results are indicative of:

a. viral infectionb. acute lymphocytic leukemiac. bacterial infectiond. normal findings

2.2. b.b. degenerative left shift – low WBC with degenerative left shift – low WBC with ↑↑bands.bands.c.c. leukemoid reaction – WBC over 50.0 K/uL.leukemoid reaction – WBC over 50.0 K/uL.d.d. leukoerythroblastic – immature neuts & NRBCs.leukoerythroblastic – immature neuts & NRBCs.

3.3. Her results are indicative of:Her results are indicative of:a.a. viral infection – lymphocytosis & reactive viral infection – lymphocytosis & reactive lymphs.lymphs.b.b. acute lymphocytic leukemia – neutropenia, acute lymphocytic leukemia – neutropenia, anemia, anemia,

thrombocytopenia and likely blast cellsthrombocytopenia and likely blast cells..c.c. bacterial infection – toxic neutrophils are bacterial infection – toxic neutrophils are present.present.d.d. normal findings – a 4 yo often has 45-75% normal findings – a 4 yo often has 45-75% lymphs.lymphs.

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Case B This 2 year old male has had multiple infections since age of 1 year. Infections have included pneumonias, skin infections andabscesses of the lung due primarily to staphylococci. His bloodsmear shows white blood cells that are normal in appearancehowever his red cells are acanthocytes.

1. What is his most likely disorder?a. Pelger-Huet anomalyb. May-Hegglin anomalyc. Alder-Reilly anomalyd. Chediak-Higashi anomalye. Chronic Granulomatous disease

1. 1. e.e. Chronic Granulomatous disease; the WBCs Chronic Granulomatous disease; the WBCs appear normal but function abnormally. CGD WBCs appear normal but function abnormally. CGD WBCs can’t produce the superoxide anion needed to kill can’t produce the superoxide anion needed to kill ingested organisms; die of staph infections. The X-ingested organisms; die of staph infections. The X-linked form linked form isis associated with McLeod phenotype and associated with McLeod phenotype and acanthocytes.acanthocytes.

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The LAP stain detects the activity of the enzyme alkalinephosphatase in the cytoplasm of neutrophils (2o granules).The amount of red precipitate in seg & band neutrophils isgraded 0-4+ to obtain a score. A normal score is 13-130. 1. A high LAP score (185) was

obtained on the LAP stain shown. This is consistent with all of the following except:a. Chronic myelocytic leukemiab. Severe bacterial infectionc. Polycythemia verad. Myelofibrosis See LAP on SL pg 1931.1. a.a. CML; a score <13 is usual. The main use of theCML; a score <13 is usual. The main use of the

LAP is to differ CML (low score) from a severe bacterial LAP is to differ CML (low score) from a severe bacterial infection/NLR (high score). The LAP is also used to differ infection/NLR (high score). The LAP is also used to differ the chronic myeloproliferative disorders; a score >130 is the chronic myeloproliferative disorders; a score >130 is typical of PV and MMM; ET usually has a normal score.typical of PV and MMM; ET usually has a normal score. P

(4+)

LAP

Case CA 50 year old male with a ‘red nose’ and chest pain has thefollowing lab data:

WBC 20.0 K/uL; Hgb 22.0 g/dL; PLT 995 L/uLDiff: 52 segs-15 bands-12 lymphs-7 monos-4 basos; 1 NRBCLAP 144; Erythropoietin (EPO) level low

1. His results are most consistent with:a. aplastic anemiab. primary polycythemia verac. idiopathic/essential thrombocythemiad. chronic myelocytic leukemiae. polycythemia secondary to hypoxiaf. pseudo polycythemia

1.1. b.b. primary polycythemia; based on pancytosis (only primary polycythemia; based on pancytosis (only disorder!!), low EPO (malignant RBC production) and disorder!!), low EPO (malignant RBC production) and high LAP score. high LAP score. See CMPD chart SL pg 205See CMPD chart SL pg 205 P

Case C2. How did you rule out the other possibilities (a,c,d,e,f)?3. What RBC mass and plasma volume is typical for this

type of patient?4. What bone marrow M:E ratio do you expect?5. What is the usual treatment?

2. a.2. a. aplastic anemia – pancytopeniaaplastic anemia – pancytopenia c.c. ET – normal/low HGB, normal LAPET – normal/low HGB, normal LAP d.d. CML – normal/low HGB, low LAPCML – normal/low HGB, low LAP e.e. polycythemia 2polycythemia 2oo to hypoxia – normal WBC & to hypoxia – normal WBC &

PLT, PLT, increased EPO, normal LAPincreased EPO, normal LAP f.f. pseudo polycythemia – normal WBC, PLT and pseudo polycythemia – normal WBC, PLT and

EPOEPO3. Increased RBC mass with normal plasma volume.3. Increased RBC mass with normal plasma volume.4.4. Normal M:E ratio; all cell lines are increased.Normal M:E ratio; all cell lines are increased.5.5. Phlebotomy to reduce blood viscosity.Phlebotomy to reduce blood viscosity. P

Case DThe following results were obtained on a 75 year old malewith severe abdominal pain:

WBC 5.0 K/uL; Hgb 8.0 g/dL; PLT 99 K/uLDiff: 16 segs-22 bands-28 lymphs-16 monos-1 eos-1 baso-

4 metas-8 myelos-4 promyelos; 30 NRBCs/diffSeveral teardrop red cells were noted. A bone marrow aspiratewas a “dry tap”. LAP 142; Philadelphia chromosome negative.

1. These results are consistent with:a. primary polycythemia rubra verab. idiopathic/primary thrombocythemiac. chronic myelocytic leukemiad. myelofibrosis with myeloid metaplasia

1.1. d.d. myelofibrosis with myeloid metaplasia; noted for a myelofibrosis with myeloid metaplasia; noted for a dry tap, leukoerythroblastic diff, teardrops on smear.dry tap, leukoerythroblastic diff, teardrops on smear.See CMPD chart SL pg 205See CMPD chart SL pg 205 P

Case D2. How did you rule out the other possibilities (a,b,c)?3. What is seen on a leukoerythroblastic differential?4. What does myeloid metaplasia mean? What was the

likely cause of this patient’s abdominal pain?

2. a. primary polycythemia vera – high HGB & PLT2. a. primary polycythemia vera – high HGB & PLT b. idiopathic/primary thrombocythemia – high PLTb. idiopathic/primary thrombocythemia – high PLT c. chronic myelocytic leukemia – high WBC, normal orc. chronic myelocytic leukemia – high WBC, normal or high PLT, low LAP, 90% of cases are Philadelphia high PLT, low LAP, 90% of cases are Philadelphia chromosome positivechromosome positive3.3. Presence of immature neutrophils (left shift) andPresence of immature neutrophils (left shift) and nucleated red cells; fibrotic bone marrow, release of nucleated red cells; fibrotic bone marrow, release of

cells is disturbed.cells is disturbed.4.4. Myeloid metaplasia = extramedullary hematopoiesis. Myeloid metaplasia = extramedullary hematopoiesis.

This patient’s pain was likely caused by an enlarged This patient’s pain was likely caused by an enlarged spleen…the spleen is often massive in MMM.spleen…the spleen is often massive in MMM. P

Case EA 60 year old female whose history includes several episodes of GI bleeding has the following lab data:

WBC 8.0 K/uL; Hgb 8.0 g/dL; PLT 2,300,000/uLDiff: 70 segs-10 bands-18 lymphs-2 monosHer blood smear is shown.

1. Her results are consistent with:a. primary polycythemia verab. myelofibrosisc. essential thrombocythemiad. reactive thrombocytosis

2. What marrow cell is increased?

1.1. c.c. essential/primary thrombocythemia; based on PLT essential/primary thrombocythemia; based on PLT count over 1 million/uL (PLT is <1 million/uL in reactive), count over 1 million/uL (PLT is <1 million/uL in reactive), giant/bizarre platelets, often with abnormal function.giant/bizarre platelets, often with abnormal function.

2.2. Increased number of megakaryocytes in the marrow.Increased number of megakaryocytes in the marrow. P

Case FA 55 year old male comes to the ER with a temperature of1030 F. His lab findings:

WBC 74.8 K/uL; Hgb 11.5 g/dL; PLT 305 K/uL; LAP 210Diff: 44 segs-40 bands-1 lymph-3 monos-0 eos-0 basos-5 metas-7 myelos. His WBC abnormalities are shown.

1. What is his most likely disorder and its cause?

2. How did you rule out CML?

1.1. Neutrophilic leukemoid Neutrophilic leukemoid reaction caused by a severe reaction caused by a severe bacterial infection; based on bacterial infection; based on WBC >50.0 K/uL, neutrophilia WBC >50.0 K/uL, neutrophilia with left shift, toxic neutrophils and LAP score >130.with left shift, toxic neutrophils and LAP score >130.

2.2. CML typically has CML typically has ↑↑eos, eos, ↑↑basos, a low LAP score and basos, a low LAP score and immature neutrophils are not ‘toxic’.immature neutrophils are not ‘toxic’. P

Case GA 61 year old farmer complained of generalized bone painto his local medical doctor . He was noted to havesplenomegaly during his physical exam. Lab data:

WBC 145.8 K/uL; Hgb 10.1 g/dL; PLT 344 K/uL; LAP 1Diff: 56 segs-15 bands-4 lymphs-1 monos-2 eos-5 basos-4 metas-12 myelos-1 promyelo. An occasional blast was noted.Chromosome analysis revealed the 9;22 translocation.

1. What is his most likely disorder? Support your answer.2. Is the presence of the Philadelphia chromosome associated

with a better prognosis? What cell lines are involved?3. What would you expect in his bone marrow?

1.1. CML; neutrophilic leukocytosis with left shift, CML; neutrophilic leukocytosis with left shift, ↑ basos, ↑ basos, LAP score <13, splenomegaly, Ph1 chromosome (+).LAP score <13, splenomegaly, Ph1 chromosome (+).

2.2. Better prognosis; present in all cells except lymphs.Better prognosis; present in all cells except lymphs.3.3. Hypercellular, high M:E ratio due to ↑↑ granulocytes.Hypercellular, high M:E ratio due to ↑↑ granulocytes. P

Case HA 43 year year old female was diagnosed with CML twoyears ago when a WBC of 121.1 K/uL was discoveredduring a routine yearly physical. She now presents withfever and the following lab data:

WBC 25.8 K/uL; Hgb 7.5 g/dL; PLT 34 K/uLHer diff has 40% blasts and chromosome analysis revealedmultiple chromosome abnormalities.

1. What has occurred? What is the prognosis?2. Under what circumstances might the LAP score in a CML

patient be greater than 13?

1.1. Blast crisis; note the severe anemia and thrombocyto-Blast crisis; note the severe anemia and thrombocyto-penia that is not typical of a chronic leukemia. The penia that is not typical of a chronic leukemia. The blasts are usually myeloid but can also be lymphoid; blasts are usually myeloid but can also be lymphoid; flow cytometry is requiredflow cytometry is required. Prognosis is generally poor.. Prognosis is generally poor.

2.2. A normal LAP is seen during remission or infection.A normal LAP is seen during remission or infection. P

Case IThis 53 year year old male had noticed the onset of easyfatigability and bruising. He saw his doctor when hedeveloped a boil in the perianal area. His CBC and subsequent bone marrow exam (shown) were abnormal. Lab data:

WBC 78.0 K/uL; Hgb 7.3 g/dLPLT 59 K/uL

1. What is the most likely diagnosis? 2. What further testing would you suggest?

1.1. Acute myeloid leukemia; based on predominance of Acute myeloid leukemia; based on predominance of blast cells (over 30%), lack of normal precursors, blood blast cells (over 30%), lack of normal precursors, blood values and the blast with an Auer rod that excludes ALL. values and the blast with an Auer rod that excludes ALL.

2.2. Cytochemical stains, immunophenotyping andCytochemical stains, immunophenotyping and chromosome analysis (most often done on marrow).chromosome analysis (most often done on marrow). P

Bone Marrow

3. What peroxidase, SBB, and PAS stain results do you expect for the blast cells in Case I? How will Auer rods stain?

4. Which of the following marker panels do you expect will be positive in this case?a. CD13, CD33, CD34b. CD2, CD3, CD4c. CD10, CD19, CD20

5. Are the expected stain & marker results always obtained?

3. Myeloid cells should stain positive for peroxidase & SBB3. Myeloid cells should stain positive for peroxidase & SBB and negative for PAS, as do Auer rods (1and negative for PAS, as do Auer rods (1oo granules) . granules) . 4.4. a. Early/immature myeloid cells should be positive for a. Early/immature myeloid cells should be positive for

CD13, CD33, CD34, and negative for lymphoid markers. CD13, CD33, CD34, and negative for lymphoid markers.

5.5. No, the “expected” is not always present. This is whyNo, the “expected” is not always present. This is why morphologic, cytochemical, cell marker, cytogenetic andmorphologic, cytochemical, cell marker, cytogenetic and clinical information must be correlated. clinical information must be correlated. Diagnosis isDiagnosis is never based on a single diagnostic tool.never based on a single diagnostic tool.

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Case JA 48 year female with a sudden onset of weakness andfatigue went to her doctor. Her lab data revealed:

WBC 33.6 K/uL; Hgb 5.3 g/dLPLT 24 K/uLDiff: 1 seg-11 lymphs-88 blastsThe blasts (shown) were noted to have some monocytic features and an occasional Auer rod. Thebone marrow showed similar cells.

1. What is the most likely diagnosis? 2. What further testing would you suggest that could determine

the leukemia subtype?

1.1. Acute myeloid leukemia…….AML, AMML, or AMonoL. Acute myeloid leukemia…….AML, AMML, or AMonoL. 2.2. Cytochemical stains and immunophenotyping areCytochemical stains and immunophenotyping are needed to determine the FAB subtype. needed to determine the FAB subtype. P

The blasts in Case J were peroxidase and SBB positiveand showed CD13, CD33, and CD34 cell markers. Thecells were also positive for CD14, a monocytic marker. The blast cells showed positivity for both the specific andnonspecific esterase stains. See SL pg 192 and Morph unitLeukemia Handouts (pink) and/or McKenzie text chapter 26.

3. Based on these results, which type of acute leukemia does this lady most likely have? Support your answer.

3.3. AMML, FAB type M4 which is characterized by cells withAMML, FAB type M4 which is characterized by cells with both granulocytic and monocytic features. The SBB, both granulocytic and monocytic features. The SBB, peroxidase, and cell marker results are consistent withperoxidase, and cell marker results are consistent with this diagnosis. this diagnosis.

The The specificspecific esterase is positive in granulocytes and esterase is positive in granulocytes and negative in monocytes. The negative in monocytes. The nonspecificnonspecific esterase is esterase is negative in granulocytes and positive in monocytes.negative in granulocytes and positive in monocytes. BothBoth esterases should be positive in cases of AMML. esterases should be positive in cases of AMML.

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Case KA 24 year old woman was admitted due to weakness and asevere nosebleed that she could not stop. Lab findingsrevealed anemia and severe thrombocytopenia (PLT 18 K/uL).

Her WBC was normal but her diff had 2 segs-40 blasts and58 blast-like cells with intense granulation. Some cells hadbundles of ‘bodies’ in the cytoplasm.

STAT coag results showed prolonged PT/APTT tests, a lowfibrinogen level, and a positive Dimer test.

1. Which type of acute leukemia do you suspect? 2. What do you think the ‘bodies’ are?3. What is the significance of her coagulation results?

1.1. Promyelocytic, M3; predominance of blasts & promyelos. Promyelocytic, M3; predominance of blasts & promyelos. 2.2. Multiple Auer rods.Multiple Auer rods.3.3. She has DIC which is a frequent presentation for AProL.She has DIC which is a frequent presentation for AProL.

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The bone marrow performed on the patient in Case K isshown. Additional testing included cytochemical stains, flow cytometry and cytogenetics.4. What results do you expect

for the peroxidase, SBB and specific esterase stains?5. What genetic abnormality

was likely detected?

4.4. All three stains would likely show brilliant positivity. All three stains would likely show brilliant positivity. A nonspecific esterase would be negative and a PAS, A nonspecific esterase would be negative and a PAS, if done, would be negative. Immunophenotyping if done, would be negative. Immunophenotyping would detect the presence of myeloid markers.would detect the presence of myeloid markers.

5.5. t(15;17), virtually diagnostic for AProL. Pt(15;17), virtually diagnostic for AProL. Prognosis is rognosis is fairly good if DIC can be controlled; patients with this fairly good if DIC can be controlled; patients with this translocation (RARA oncogene) respond well to translocation (RARA oncogene) respond well to retinoic acid….causes maturation of promyelocytes.retinoic acid….causes maturation of promyelocytes. P

Bone Marrow

Case LAn 80 year old man with abnormal blood and bone marrowfindings is suspected to have acute erythroleukemia (EL), M6. His bone marrow contains 40% myeloblasts and numerousgiant RBC precursors similar to those seen in megaloblasticanemia, however his folate and B12 levels are normal.

1. What term is used to describe giant RBC precursors that mimic those seen in megaloblastic anemia? See Morph unit Terms Handout (lavender).

2. What cytochemical stain is used to differentiate malignant erythroid cells from normal red cell precursors?See SL pg 192 and/or Morph unit Leukemia Handouts (pink).

1.1. MegaloblastMegaloblastoidoid red cells; resemble megaloblastic RBCs red cells; resemble megaloblastic RBCs but are caused by abnormal growth and associated with but are caused by abnormal growth and associated with malignancy such as EL and myelodysplastic syndromes.malignancy such as EL and myelodysplastic syndromes.

2.2. The PAS stain; normal erythrocytes are negative and The PAS stain; normal erythrocytes are negative and malignant red cells stain PAS positive.malignant red cells stain PAS positive. P

The giant, multinucleated RBC precursors from the patient in Case L (shown on the right) stained PAS positive (on left). This is consistent with a diagnosis of EL, FAB type M6.

Erythroleukemia behaves similar to CML cases that Erythroleukemia behaves similar to CML cases that develop a ‘blast crisis’. The bone marrow in the develop a ‘blast crisis’. The bone marrow in the terminal stage of EL shows mainly myeloblasts.terminal stage of EL shows mainly myeloblasts.

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Case MAn 70 year old female with “possible leukemia” is transferred toyour hospital for evaluation. A CBC reveals the following:

WBC 2.4 K/uL; Hgb 9.0 g/dL; MCV 110 fL; RDW 22.4%;PLT 80 K/uL; Diff: 68 segs-6 bands-18 lymphs-3 monos-5 blasts

Her folate and B12 levels are normal. A bone marrow examreveals 75% cellularity and 24% blasts, a few with Auer rods.Erythrocytic, granulocytic and megakaryocytic precursorsexhibit features of abnormal growth. Monocytic cells are normalin number. See Morph unit MDS Handout (lavender)

1. According to the FAB classification, this patient likely has:a. AML, M2b. Refractory Anemia with Excess Blasts (RAEB)c. RAEB in transformation (RAEB-T)d. ALL, L2

1.1. c. RAEB in transformation c. RAEB in transformation P

2. You suspect this diagnosis for Case M because…………3. Does this patient exhibit one or more blood cytopenias?4. Name at least five signs of abnormal growth that were most

likely present in the bone marrow (or blood) of this patient.5. According to the WHO classification, this patient has:

2.2. Her bone marrow is hypercellular with 24% blasts & Auer Her bone marrow is hypercellular with 24% blasts & Auer rods and 5% blasts in her blood. Criteria for RAEB-T is rods and 5% blasts in her blood. Criteria for RAEB-T is 20-30% blasts in BM and 5% or more blasts in the blood. 20-30% blasts in BM and 5% or more blasts in the blood. She does not fit the FAB criteria for AML (>30% blasts).She does not fit the FAB criteria for AML (>30% blasts).

3.3. Multiple cytopenias; low WBC, low PLT and a macrocytic Multiple cytopenias; low WBC, low PLT and a macrocytic anemia not due to low folate or Banemia not due to low folate or B1212. Her high RDW may . Her high RDW may

be caused by a dimorphic RBC population.be caused by a dimorphic RBC population.4.4. Multinucleated and/or megaloblastMultinucleated and/or megaloblastoidoid RBCs, ringed RBCs, ringed

sideroblasts, hyposegmented (aka pelgersideroblasts, hyposegmented (aka pelgeroidoid)) and/or and/or hypogranular neutrophils, micromegakaryocytes. hypogranular neutrophils, micromegakaryocytes. See next slide to view features of abnormal growth.See next slide to view features of abnormal growth.

5.5. AML; the change to 20% blasts has eliminated RAEB-T. AML; the change to 20% blasts has eliminated RAEB-T. P

Case M P

Normal rubricytes Megaloblastoid rubricytes Multinucleated RBCs

MicromegakaryocyteHypogranular neutrophil

Case NA 6 year old boy had a 2 to 3 week history of fever at nightand cervical adenopathy. Physical exam showed ageneralized lymphadenopathy. His lab data:

WBC 20.7 K/uL; Hgb 10.9 g/dL; PLT 123 K/uLDiff results: 1 segs-17 lymphs-1 monos-81 blasts

1. What do you suspect?2. What is his absolute neutrophil count? Are you

concerned?3. What is the next step for this patient?

1.1. Acute leukemia, probably ALL; based on his age, the Acute leukemia, probably ALL; based on his age, the predominance of blasts but no Auer rods noted.predominance of blasts but no Auer rods noted.

2.2. Critically low ANC of 207/uL; severe neutropenia. Critically low ANC of 207/uL; severe neutropenia. 3.3. Bone marrow aspirate/biopsy & spinal tap; staining Bone marrow aspirate/biopsy & spinal tap; staining

panel, cell markers and cytogenetics.panel, cell markers and cytogenetics. P

The bone marrow obtained on the patient in Case N was hypercellular with 80% blasts that were fairly small and homogeneous. Blasts were not detected in his CSF. His blasts were peroxidase/SBB negative and PAS positive. Immunophenotyping showed positivity for CD10, CD19/20, and CD34; cells were negative for CD2/3. See Morph unit Leukemia Handouts (pink) and/or McKenzie text.

4. These results are most consistent with: a. Precursor-B ALL, CALLA type b. T cell ALL5. Does this patient have a good prognosis?

4.4. a. Precursor-B ALL (probably FAB L1 morphology).a. Precursor-B ALL (probably FAB L1 morphology).ALL-L3 is always a B cell malignancy and is known ALL-L3 is always a B cell malignancy and is known as the Burkitt’s type. The FAB types L1 & L2 are far as the Burkitt’s type. The FAB types L1 & L2 are far less important than the immunophenotype. less important than the immunophenotype.

5.5. A child with CALLA type has a good prognosis.A child with CALLA type has a good prognosis. P

Case OThis 72 year old male was discovered to have an elevatedWBC count 4 years ago. He has never received therapyfor his disorder. Physical examination showed no palpablelymphadenopathy. Lab data:

WBC 50.7 K/uL; Hgb 13.9 g/dLPLT 223 K/uLDiff:10 segs-89 lymphs-1 monoHis blood smear is shown.

1. What is his most likely diagnosis and why?2. Are these usually malignant T or B cells?

1.1. CLL, based on age, normal HGB/PLT, predominance of CLL, based on age, normal HGB/PLT, predominance of mature lymphs with hyperclumped nuclear chromatin mature lymphs with hyperclumped nuclear chromatin & smudge cells.& smudge cells.

2.2. Almost all cases of CLL are B cell type.Almost all cases of CLL are B cell type. P

Case PA 75 year old male presents with pancytopenia andsplenomegaly. Several ‘lymph-like’ cells with monocyticcytoplasm are observed on his blood smear. A bone marrow aspirate is a “dry tap”. A TRAP stain, done on the ‘lymph-like’ cells, is shown.

1. This information is consistent with a diagnosis of: a. Hodgkin’s lymphomab. CMV infectionc. Hairy cell leukemiad. Chronic lymphocytic leukemia in blast crisise. Waldenstrom’s macroglobulinemia

1.1. c. HCL; hairy cells stain c. HCL; hairy cells stain acidacid phosphatase positive phosphatase positive and are resistant to tartrate inhibition (stay positive). and are resistant to tartrate inhibition (stay positive). The splenomegaly and dry tap BM are usual findings.The splenomegaly and dry tap BM are usual findings. P

TRAP

The non-Hodgkin’s lymphomas consist of a very largegroup of disorders that are constantly being reclassified.You are responsible for selected examples. Refer to SL pg 207 and pg 7 of the Rotation II Morph unit objectives.

NHL comments:a. Patients with NHL often have a normal CBC/diff initially. b. A lymphoma and a leukemia with the same malignant cell

type are the same disease, they just have a different presentation, i.e. marrow and blood vs lymphoid tissue.

c. Immunophenotyping is essential for determining cell maturity and cell type; most NHL’s are B cell type.

See the next slides to view the malignant cell types inSee the next slides to view the malignant cell types incertain non-Hodgkin’s lymphomas.certain non-Hodgkin’s lymphomas.

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Patient A has small lymphocytic lymphoma that has spread from the tissues to the bone marrow and blood. SLL is the tissue counterpart of:Patient B has peripheralized ‘mixed’ cell lymphocytic lymphoma. These clefted lymphoid cells are called:

▪▪SLL is tissue CLL; patient A has small, hyperclumped SLL is tissue CLL; patient A has small, hyperclumped lymphs. lymphs. ▪▪Patient B has clefted cells called Rieder cells.Patient B has clefted cells called Rieder cells.

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Patient BPatient A

Patient C has peripheralized Burkitt’s lymphoma that is associated with EBV and t(8;14). The leukemic form is called: Patient D has mycosis fungoides, a T cell lymphoma that involves the:

▪▪Patient C has B cell-ALL, L3. Patient C has B cell-ALL, L3. Burkitt cells have fat vacuoles Burkitt cells have fat vacuoles in the nucleus & cytoplasm.in the nucleus & cytoplasm.▪▪Patient D has a T cell lymphoma of the skin (note red skin Patient D has a T cell lymphoma of the skin (note red skin and ulcers); the leukemic form is called Sezary syndrome.and ulcers); the leukemic form is called Sezary syndrome. P

Patient D

Skin biopsy Sezary cells

Patient C

Identify the cell

A MONKEY A MONKEY A MONKEY A MONKEY